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Type C (type + c)
Kinds of Type C Selected AbstractsAltered apolipoprotein E glycosylation is associated with A,(42) accumulation in an animal model of Niemann-Pick Type C diseaseJOURNAL OF NEUROCHEMISTRY, Issue 6 2010Ching-Ching Chua J. Neurochem. (2010) 112, 1619,1626. Abstract Neurodegeneration is the final cause of death in Niemann-Pick Type C (NPC) disease, a cholesterol-storage disorder. Accumulating evidence indicates that NPC may share common pathological mechanisms with Alzheimer's disease, including the link between aberrant cholesterol metabolism and amyloid-, (A,) deposition. Apolipoprotein E (apoE) is highly expressed in the brain and plays a pivotal role in cholesterol metabolism. ApoE can also modulate A, production and clearance, and it is a major genetic risk factor for Alzheimer's disease. Although apoE is glycosylated, the functional significance of this chemical alteration on A, catabolism is unclear. In this study using an NPC animal model, we detect specific changes in apoE glycosylation that correlate with increased A,(42) accumulation prior to the appearance of neurological abnormalities. This suggests that increased apoE expression could be a compensatory response to the increased A,(42) deposition in NPCnih mice. We also observe what appears to be a simplification of the glycosylation process on apoE during neurodegeneration. [source] Reversible Cytotoxic Edema in a Cirrhotic Patient Following TIPSJOURNAL OF NEUROIMAGING, Issue 4 2009James R. Babington MD ABSTRACT The authors report the magnetic resonance imaging (MRI) findings in a 52-year-old man with cirrhosis from chronic hepatitis C who developed episodic acute hepatic encephalopathy Type C following placement of transjugular intrahepatic portosystemic shunt (TIPS). Brain MRI revealed hyperintense T2 signal and restricted diffusion distributed through the cerebral cortex. The patient's mentation improved with treatment of his hyperammonemia. Brain MRI performed 5 months later revealed diffuse cerebral atrophy and new areas of hyperintense T2 signal in the cerebral white matter. The cortical signal abnormalities and low apparent diffusion coefficient values on the initial MRI resolved with exception of a mild amount of hyperintense FLAIR signal in the cingulate cortex. Acute hepatic encephalopathy following portosystemic shunting,either from placement of TIPS or from development of spontaneous shunts,is a widely recognized complication of portal hypertension and cirrhosis. We report MRI findings of reversible cytotoxic edema in a patient with acute hepatic encephalopathy following placement of TIPS. [source] A Clinicopathological Study of Postoperatively Upgraded Early Squamous-Cell Carcinoma of the Uterine CervixJOURNAL OF OBSTETRICS AND GYNAECOLOGY RESEARCH (ELECTRONIC), Issue 4 2000Dr. Manabu Yoshida Abstract Objective: To investigate the clinicopathological backgrounds and diagnostic problems of postoperatively upgraded early squamous-cell carcinomas of the uterine cervix Patients and Methods: A total of 23 patients with postoperatively upgraded early squamous-cell carcinomas who were treated at the Saitama Cancer Center during the period of January 1, 1976, through December 31, 1991, were analyzed clinicopathologically. We reexamined the Pap smears (ectocervix, endocervix), colposcopic findings, punch biopsies, and histological findings of the operative specimens. All patients were divided into one of 3 groups based on each patient's main location of the carcinoma of the cervix: Type A: ectocervical type; Type B: endocervical type; or Type C: combined (ectocervical and endocervical) type. Clinical staging of the uterine cervical carcinomas was done in accordance with the 1994 FIGO rules. Results: The numbers of patients were: Type A, 2; Type B, 10; Type C, 11. Of the 23 patients, 21 (91.3%) had lesions in the endocervical portion at least. Fifteen patients (65.2%) complained of atypical vaginal bleeding. Colposcopic findings suggesting an invasive carcinoma appeared for only 6 patients (26.1%). A cytological reevaluation revealed that the endocervical findings were much stronger than the ectocervical ones in 10 (66.7%) of 15 patients whose smears of both sites could be rechecked. Conclusions: Even if the preoperative diagnosis was early cervical carcinoma, CIS or Stage Ia1, the signs of atypical vaginal bleeding suggested that the final clinical stage would be upgraded after an operation. Furthermore, when the endocervical cytological findings were much more exaggerated than the ectocervical ones, the possibility of deeply invaded endocervical lesions should be considered. [source] Mitotic Epitopes are Incorporated into Age-dependent Neurofibrillary Tangles in Niemann,Pick Disease Type CBRAIN PATHOLOGY, Issue 2 2010Min Zhang MD Abstract The mechanism underlying neurofibrillary tangles (NFTs) in Alzheimer's disease (AD) and other neurodegenerative disorders remains elusive. Niemann,Pick disease type C (NPC) is a kind of genetic neurovisceral disorder in which the intracellular sequestration of cholesterol and other lipids in neurons, NFT formation and neuronal degeneration in brain are the neuropathology hallmarks. The age of onset and progression of the disease vary dramatically. We have analyzed the hippocampus from 17 NPC cases, aged from 7 months to 55 years, to depict the temporal characteristics of NFT formation. Unexpectedly, classic NFT was observed in about 4-year-old NPC brain, suggesting that NFT is not aging dependent, and that juvenile brain neurons satisfy the requirements for NFT formation. NFT in the hippocampus of NPC was significantly increased in number with the advance of age. More importantly, multiple mitotic phase markers, which are not usually found in normal mature neurons, were abundant in the affected neurons and incorporated into NFT. The unusual activation of cdc2/cyclin B kinase and downstream mitotic indices are closely associated with the age-dependent NFT formation, signifying the contribution of abortive cell cycle to neurodegeneration. The cdc2 inhibitors may be therapeutically used for early intervention of neurodegeneration and NFT formation in NPC. [source] Structural, electronic, and optical properties of 9-heterofluorenes: A quantum chemical studyJOURNAL OF COMPUTATIONAL CHEMISTRY, Issue 13 2007Run-Feng Chen Abstract Density-functional theory studies were applied to investigate the structural, electronic, and optical properties of 9-heterofluorenes achieved by substituting the carbon at 9 position of fluorene with silicon, germanium, nitrogen, phosphor, oxygen, sulfur, selenium, or boron. These heterofluorenes and their oligomers up to pentamers are highly aromatic and electrooptically active. The alkyl and aryl substituents of the heteroatom have limited influence, but the oxidation of the atom has significant influence on their molecular structures and properties. The highest occupied molecular orbital (HOMO)-lowest occupied molecular orbital (LUMO) interaction theory was successfully applied to analyze the energy levels and the frontier wave functions of these heterofluorenes. Most heterofluorenes belong to type B of interaction with low-lying LUMO and have the second kind of wave function. Carbazole and selenafluorene have type C of interaction with high-lying HOMO and the third kind of wave function. Types C and D of heterofluorenes, such as carbazole, oxygafluorene, sulfurafluorene, and selenafluorene also have high triplet state energies. The extrapolated HOMO and LUMO for polyheterofluorenes indicate that polyselenonafluorene has the lowest LUMO; polycarbazole has the highest HOMO; polyselenafluorene has the highest bandgap (Eg); and polyborafluorene has the lowest Eg. Heterofluorenes and their oligomers and polymers are of great experimental interests, especially those having extraordinary properties revealed in this study. © 2007 Wiley Periodicals, Inc. J Comput Chem, 2007 [source] Pseudotracheal tubes, larval head, and mycophagy in Sepedophilus (Coleoptera: Staphylinidae: Tachyporinae)JOURNAL OF ZOOLOGICAL SYSTEMATICS AND EVOLUTIONARY RESEARCH, Issue 1-2 2001Leschen Newton (in: Wheeler, Q.; Blackwell, M. (eds), Fungus-Insect Relationships: Perspectives in Ecology and Evolution. New York: Columbia University Press, pp. 302–353, 1984) characterized five types of Sepedophilus larvae based on head structures and the external and internal features of the head of larvae of Sepedophilus type C are described in detail herein. A functional interpretation of structures involved with feeding is made on the basis of morphological and behavioural observations. Types C and D larvae possess tube-like epipharyngeal structures resembling dipteran labial pseudotrachea, which may play an important role in a specialized liquid-feeding process. Based on a preliminary analysis of head characters delimited by Newton (1984) it is shown that mycophagy has evolved once from a predatory ancestor, although some Sepedophilus groups may have mixed feeding strategies. The epipharyngeal tubes are demonstrated to be unique to mycophagous Sepedophilus in Coleoptera whereas the overall head structure is very similar to mycophagous larvae in the family Sphindidae. Pseudotracheen, Larvale Kopfstrukturen und Mycetophagie bei Sepedophilus (Coleoptera: Staphylinidae: Tachyporinae) Newton (in: Wheeler, Q.; Blackwell, M. (eds), Fungus-Insect Relationships: Perspectives in Ecology and Evolution. New York: Columbia University Press, pp. 302–353, 1984) charakterisierte fünf Larventypen von Sepedophilus Gistel, 1856 basierend auf abweichenden Kopfstrukturen. In der vorliegenden Arbeit werden äußere und innere Merkmale des Kopfes von Larven des Typs C detailliert beschrieben. Eine funktionelle Interpretation der am Freßvorhang beteiligten Kopfstrukturen beruht auf morphologischen Befunden und Lebendbeobachtungen. Larven der Typen C und D besitzen röhrenförmige epipharyngeale Strukturen, die an labiale Pseudotracheen von Dipteren erinnern. Sie spielen wahrscheinlich eine wichtige Rolle bei der Ernährung von verflüssigtem Substrat. Eine vorläufige Analyse von Kopfmerkmalen, die von Newton (1984) definiert wurden, ergibt ein nur einmaliges Entstehen von Mycetophagie ausgehend wahrscheinlich von räuberischen Vorfahren. Dabei kann allerdings nicht ausgeschlossen werden, daßSepedophilus -Larven auch unterschiedliche Nahrungsresourcen nutzen können. Die epipharyngealen Röhren kommen außer bei mycetophagen Sepedophilus -Arten bei keiner anderen Coleopterengruppe vor. Die übrigen Kopfstrukturen erinnern dagegen stark an mycetophage Larven der Familie Sphindidae. [source] Preliminary study of mucosal IgA in the equine small intestine: specific IgA in cases of acute grass sickness and controlsEQUINE VETERINARY JOURNAL, Issue 5 2007F. G. NUNN Summary Reasons for performing study: There is much evidence to suggest that group III Clostridium botulinum (types C and D) are involved in the aetiology of equine grass sickness (EGS). Antibodies have been detected previously in the blood and high levels associated with resistance to disease. Specific mucosal antibodies in the gastrointestinal (GI) tract are likely to be important in protection, and this study was performed to ascertain if such antibodies could be detected and if their levels were related to disease state. Objectives: To develop a method for quantifying IgA antibodies to C. botulinum types C and D in the GI tract of horses and to relate antibody levels to disease status. Methods: Samples of tissue (n = 25: 6 duodenum, 7 jejunum and 12 ileum) were taken from acute grass sickness (AGS) cases and from control horses (n = 12; 4 samples from each site) at post mortem. They were extracted with the detergent saponin in the presence of protease inhibitors and assayed for total IgA, for specific IgA against botulinum neurotoxins types C and D (BoNT/C or BoNT/D), and against surface antigens of a BoNT/C negative strain of C. botulinum type C (SA) and of Clostridium tetani (TetSA), as a control. Specific IgA was expressed as percentage total IgA. Results: Compared to controls, significantly higher levels of specific IgA against BoNT/C were detected in the jejunum (P = 0.04) and ileum (P = 0.02) of AGS cases. Similarly, higher specific levels against BoNT/D were demonstrated in duodenum (P = 0.01) and jejunum (P = 0.02). Significantly higher levels of IgA against SA were demonstrated only in duodenal samples (P = 0.01). Conclusions: Levels of IgA antibody to BoNTs in control horses were at near undetectable levels, suggesting no recent exposure to toxins. In AGS cases, significantly higher levels of specific IgA were detected predominantly in jejunum and ileum. Potential relevance: If specific IgA is protective then any successful vaccine for EGS should induce a mucosal response. [source] HCV RNA-dependent RNA polymerase replicates in vitro the 3, terminal region of the minus-strand viral RNA more efficiently than the 3, terminal region of the plus RNAFEBS JOURNAL, Issue 22 2001Sandrine Reigadas The NS5B protein, or RNA-dependent RNA polymerase of the hepatitis virus type C, catalyzes the replication of the viral genomic RNA. Little is known about the recognition domains of the viral genome by the NS5B. To better understand the initiation of RNA synthesis on HCV genomic RNA, we used in vitro transcribed RNAs as templates for in vitro RNA synthesis catalyzed by the HCV NS5B. These RNA templates contained different regions of the 3, end of either the plus or the minus RNA strands. Large differences were obtained depending on the template. A few products shorter than the template were synthesized by using the 3, UTR of the (+) strand RNA. In contrast the 341 nucleotides at the 3, end of the HCV minus-strand RNA were efficiently copied by the purified HCV NS5B in vitro. At least three elements were found to be involved in the high efficiency of the RNA synthesis directed by the HCV NS5B with templates derived from the 3, end of the minus-strand RNA: (a) the presence of a C residue as the 3, terminal nucleotide; (b) one or two G residues at positions +2 and +3; (c) other sequences and/or structures inside the following 42-nucleotide stretch. These results indicate that the 3, end of the minus-strand RNA of HCV possesses some sequences and structure elements well recognized by the purified NS5B. [source] cagA gene variants in Malaysian Helicobacter pylori strains isolated from patients of different ethnic groupsFEMS IMMUNOLOGY & MEDICAL MICROBIOLOGY, Issue 2 2005Mohamed Ramelah Abstract Helicobacter pylori infection of a distinct subtype of cagA may lead to different pathological manifestation. The aim of this study is to determine the presence of cagA gene and its variants in H. pylori infection among different ethnic groups and its effect on gastroduodenal diseases. Overall detection of cagA among the 205 clinical isolates of H. pylori was 94%. Variations in size of the 3, region of cagA gene were examined among 192 Malaysian H. pylori cagA -positive strains. Results showed that three cagA variants differing in fragment length of PCR products were detected and designated as type A (621,651 bp), type B (732,735 bp) and type C (525 bp). Although there was no association between any of the cagA subtypes with peptic ulcer disease (p > 0.05), an association between cagA subtypes with a specific ethnic group was observed. Specific- cagA subtype A strains were predominantly isolated from Chinese compared to Malays and Indians (p < 0.0005), and cagA subtype B strains were predominantly isolated from Malays and Indians compared to Chinese (p < 0.05). The cagA type A strains of H. pylori is commonly found in the Chinese patients who have a higher risk of peptic ulcer disease, thus indicating that it could be used as an important clinical biomarker for a more severe infection. [source] Diagenesis of the Amposta offshore oil reservoir (Amposta Marino C2 well, Lower Cretaceous, Valencia Trough, Spain)GEOFLUIDS (ELECTRONIC), Issue 3 2010E. PLAYÀ Abstract Samples from the Amposta Marino C2 well (Amposta oil field) have been investigated in order to understand the origin of fractures and porosity and to reconstruct the fluid flow history of the basin prior, during and after oil migration. Three main types of fracture systems and four types of calcite cements have been identified. Fracture types A and B are totally filled by calcite cement 1 (CC1) and 2 (CC2), respectively; fracture type A corresponds to pre-Alpine structures, while type B is attributed to fractures developed during the Alpine compression (late Eocene-early Oligocene). The oxygen, carbon and strontium isotope compositions of CC2 are close to those of the host-rock, suggesting a high degree of fluid-rock interaction, and therefore a relatively closed palaeohydrogeological system. Fracture type C, developed during the Neogene extension and enlarged by subaerial exposure, tend to be filled with reddish (CS3r) and greenish (CS3g) microspar calcite sediment and blocky calcite cement type 4 (CC4), and postdated by kaolinite, pyrite, barite and oil. The CS3 generation records lower oxygen and carbon isotopic compositions and higher 87Sr/86Sr ratios than the host-limestones. These CS3 karstic infillings recrystallized early within evolved-meteoric waters having very little interaction with the host-rock. Blocky calcite cement type 4 (CC4 generation) has the lowest oxygen isotope ratio and the most radiogenic 87Sr/86Sr values, indicating low fluid-rock interaction. The increasingly open palaeohydrogeological system was dominated by migration of hot brines with elevated oxygen isotope ratios into the buried karstic system. The main oil emplacement in the Amposta reservoir occurred after the CC4 event, closely related to the Neogene extensional fractures. Corrosion of CC4 (blocky calcite cement type 4) occurred prior to (or during) petroleum charge, possibly related to kaolinite precipitation from relatively acidic fluids. Barite and pyrite precipitation occurred after this corrosion. The sulphur source associated with the late precipitation of pyrite was likely related to isotopically light sulphur expelled, e.g. as sulphide, from the petroleum source rock (Ascla Fm). Geofluids (2010) 10, 314,333 [source] Genetic and cellular studies of oxidative stress in methylmalonic aciduria (MMA) cobalamin deficiency type C (cblC) with homocystinuria (MMACHC),HUMAN MUTATION, Issue 11 2009Eva Richard Abstract Methylmalonic aciduria (MMA) cobalamin deficiency type C (cblC) with homocystinuria (MMACHC) is the most frequent genetic disorder of vitamin B12 metabolism. The aim of this work was to identify the mutational spectrum in a cohort of cblC -affected patients and the analysis of the cellular oxidative stress and apoptosis processes, in the presence or absence of vitamin B12. The mutational spectrum includes nine previously described mutations: c.3G>A (p.M1L), c.217C>T (p.R73X), c.271dupA (p.R91KfsX14), c.331C>T (p.R111X), c.394C>T (p.R132X), c.457C>T (p.R153X), c.481C>T (p.R161X), c.565C>A (p.R189S), and c.615C>G (p.Y205X), and two novel changes, c.90G>A (p.W30X) and c.81+2T>G (IVS1+2T>G). The most frequent change was the known c.271dupA mutation, which accounts for 85% of the mutant alleles characterized in this cohort of patients. Owing to its high frequency, a real-time PCR and subsequent high-resolution melting (HRM) analysis for this mutation has been established for diagnostic purposes. All cell lines studied presented a significant increase of intracellular reactive oxygen species (ROS) content, and also a high rate of apoptosis, suggesting that elevated ROS levels might induce apoptosis in cblC patients. In addition, ROS levels decreased in hydroxocobalamin-incubated cells, indicating that cobalamin might either directly or indirectly act as a scavenger of ROS. ROS production might be considered as a phenotypic modifier in cblC patients, and cobalamin supplementation or additional antioxidant drugs might suppress apoptosis and prevent cellular damage in these patients. Hum Mutat 30:1,9, 2009. © 2009 Wiley-Liss, Inc. [source] Impact of selected inborn errors of metabolism on prenatal and neonatal developmentIUBMB LIFE, Issue 6 2010Sabine Illsinger Abstract In general, data regarding maturational processes of different metabolic pathways in the very vulnerable fetal and neonatal period are rare. This review is to substantiate the impact of selected inborn errors of metabolism on this critical period of life and their clinical manifestation. Significant adaptation of mitochondrial/energy-, carbohydrate-, lysosomal-, and amino acid-metabolism occurs during early prenatal and neonatal development. In utero, metabolic environment has an impact on the development of the fetus as well as fetal organ maturation. Defects of distinct metabolic pathways could therefore already be of significant relevance in utero and for clinical manifestations in the early fetal and neonatal period. Disturbances of these pathways may influence intrauterine growth and health. Production of a toxic intrauterine milieu, energy-deficiency, modification of membrane function, or disturbance of the normal intrauterine expression of genes may be responsible for fetal compromise and developmental disorders. Three categories of metabolic disorders will be discussed: the "intoxication type" (classical galactosemia, ornithine transcarbamylase deficiency, and "maternal phenylketonuria"), the "storage type" (Morbus Niemann Pick type C), and the "energy deficient type" (including long-chain fatty acid oxidation disorders, pyruvate dehydrogenase deficiency, and respiratory chain defects). For these disorders, the pathophysiology of early manifestation, special aspects regarding the prenatal and neonatal period, and diagnostic as well as therapeutic options are presented. © 2010 IUBMB IUBMB Life, 62(6): 403,413, 2010 [source] Structural, electronic, and optical properties of 9-heterofluorenes: A quantum chemical studyJOURNAL OF COMPUTATIONAL CHEMISTRY, Issue 13 2007Run-Feng Chen Abstract Density-functional theory studies were applied to investigate the structural, electronic, and optical properties of 9-heterofluorenes achieved by substituting the carbon at 9 position of fluorene with silicon, germanium, nitrogen, phosphor, oxygen, sulfur, selenium, or boron. These heterofluorenes and their oligomers up to pentamers are highly aromatic and electrooptically active. The alkyl and aryl substituents of the heteroatom have limited influence, but the oxidation of the atom has significant influence on their molecular structures and properties. The highest occupied molecular orbital (HOMO)-lowest occupied molecular orbital (LUMO) interaction theory was successfully applied to analyze the energy levels and the frontier wave functions of these heterofluorenes. Most heterofluorenes belong to type B of interaction with low-lying LUMO and have the second kind of wave function. Carbazole and selenafluorene have type C of interaction with high-lying HOMO and the third kind of wave function. Types C and D of heterofluorenes, such as carbazole, oxygafluorene, sulfurafluorene, and selenafluorene also have high triplet state energies. The extrapolated HOMO and LUMO for polyheterofluorenes indicate that polyselenonafluorene has the lowest LUMO; polycarbazole has the highest HOMO; polyselenafluorene has the highest bandgap (Eg); and polyborafluorene has the lowest Eg. Heterofluorenes and their oligomers and polymers are of great experimental interests, especially those having extraordinary properties revealed in this study. © 2007 Wiley Periodicals, Inc. J Comput Chem, 2007 [source] Fruit dispersal and seed banks in Atriplex sagittata: the role of heterocarpyJOURNAL OF ECOLOGY, Issue 2 2001Bohumil Mandák Summary 1Atriplex sagittata, an arly succesional, annual species of disturbed habitats in Central Europe, produces three types of fruits. We studied the differences in dispersal between the fruit types in order to investigate their ecological roles. 2The typical positive relationship between dispersal ability and germinability is not found in this species. Type A (ebracteate) fruits produced deeply dormant seeds and had the lowest dispersal potential, but of the two fruits with bracts, type B, with dormant seeds, was better dispersed by both water and wind than type C, which produces non-dormant seeds. 3Wind, temperature and precipitation have significant effects on fruit release but their effects differ between fruit types. The release of fruit types with bracts was positively correlated with wind whereas all fruit types tended to be negatively correlated with precipitation and temperature range. 4Type C fruit, which contains non-dormant seed, are absent from the soil in summer and have a Type II transient seed bank. Type A and B fruits, containing dormant seeds, form a persistent seed bank. 5Heterocarpy, where fruit types show distinct ecological behaviour, enables colonizing species such as A. sagittata to survive both major disturbance (by ensuring that some seeds persist) and unfavourable conditions (by spreading germination over a long period). 6In A. sagittata, seed dynamics can be explained by the germination behaviour of seeds produced by the three types of fruit. All fruit types mature in autumn, but few of Type A fall from the mother plant until spring, when germination is probably inhibited because of insufficient stratification. Type C fruit, however, show peak dispersal in winter and the majority of these non-dormant seeds are able to germinate as soon as conditions become more suitable. [source] GB virus type C infection in hemodialysis patients considering co-infection with hepatitis C virusJOURNAL OF MEDICAL VIROLOGY, Issue 7 2008S.M. Hosseini-Moghaddam Abstract GB virus type C is a well-known viral agent with capability of infecting patients undergoing hemodialysis. Liver enzyme levels in infected individuals have been reported to remain within the normal range. Simultaneous infection of GBV-C and other viral agents may occur due to common routes of transmission. A total of 104 hemodialysis patients living in Tehran were included in this case-control study (53 patients with HCV infection, group I; and 51 with no HCV infection, group II). Diagnosis was made by detection Anti-E2 protein using ELISA and HCV,RNA using RT-PCR. History of HBV-infection, organ transplantation, depression, malignancies, chemotherapy, diabetes mellitus, thyroid disorders and chronic cutaneous disorders were considered. Patients were evaluated for high- risk behaviors such as intravenous drug injection, addiction or substance abuse. A total of 14 patients (13.6%) were GBV-C-infected. Four of them were co-infected with HCV. All patients with GBV-C infection had viral genotype 2. Thirteen patients (12%) had a history of multiple blood transfusions. Mean (±SD) age of GBV-C-infected patients was 48.7,±,13.8 years. Among GBV-C infected patients, three patients had a history of organ transplantation and three had a co-morbidity of diabetes mellitus. This study as the first case-control study to evaluate the association between GBV-C and HCV infection, to our knowledge, shows hemodialysis patients living in Tehran are infected with GBV-C with intermediate level of frequency. The association of GBV-C transmission with other viral blood-borne agents might be necessary. J. Med. Virol. 80: 1260,1263, 2008. © 2008 Wiley-Liss, Inc. [source] Regional and Developmental Expression of the Npc1 mRNA in the Mouse BrainJOURNAL OF NEUROCHEMISTRY, Issue 3 2000A. Prasad Abstract: Niemann-Pick type C (NP-C) disease is a fatal, autosomal recessive disorder of cholesterol metabolism that results in progressive central nervous system deterioration and premature death. Recently, a gene mutated in NP-C disease (NPC1) was identified in both human patients and in the npcnih mouse model. Although the function of the NPC1 gene is at present unknown, determining the pattern of its expression in the brain may facilitate identification of the mechanisms underlying the neuropathology of this disease and in identifying relevant targets for any potential therapeutic intervention. We have used in situ hybridization techniques to characterize the pattern of Npc1 mRNA expression in both the wild-type and the npcnih mutant mouse brain. In adult animals of both genotypes, the Npc1 mRNA was detected in the majority of neurons in nearly all regions, but at significantly higher levels in the cerebellum and in specific pontine nuclei. Analysis of Npc1 mRNA levels during development in the wild-type mouse indicated that this transcript was expressed in neurons as early as embryonic day 15 and that a significant region-specific pattern of expression was established by postnatal day 7. Our data suggest that whereas the NPC1 gene is widely expressed in neurons of the brain, the higher levels of expression in the cerebellum and pontine structures established by early postnatal ages may make these regions more susceptible to neuronal dysfunction in NP-C disease. [source] Crystal structure and polarized vibrational spectra of 2-bromo-4-nitropyridine N -oxide single crystalJOURNAL OF RAMAN SPECTROSCOPY, Issue 4 2002J. Hanuza 2-Bromo-4-nitropyridine N -oxide is orthorhombic, space group Pbca, with eight molecules per unit cell of dimensions a = 5.979(1), b = 9.899(1), c = 23.249(5) Å. The Br ion is nearly coplanar with the pyridine ring, while the two oxygen atoms of the nitro group are displaced above and below the ring by (,0.214 Å) and (+0.053 Å). The hydrogen bond of the type C,H···O links the molecules into pairs around centers of symmetry. These dimers, arranged into layers related by glide planes, are held together solely by contacts of the van der Waals type. The polarized Fourier transform IR and Raman spectra, measured in the regions 30,3500 and 80,3500 cm,1, respectively, are correlated with x-ray structural data. Comparison of the spectrum of the dissolved sample with the spectra obtained from the polycrystalline sample and single crystals shows the attractive character of the intermolecular C,H···O contacts for these molecules. The temperature-dependent IR spectra suggest the presence of orientational disorder at higher temperatures. Copyright © 2002 John Wiley & Sons, Ltd. [source] Pseudotracheal tubes, larval head, and mycophagy in Sepedophilus (Coleoptera: Staphylinidae: Tachyporinae)JOURNAL OF ZOOLOGICAL SYSTEMATICS AND EVOLUTIONARY RESEARCH, Issue 1-2 2001Leschen Newton (in: Wheeler, Q.; Blackwell, M. (eds), Fungus-Insect Relationships: Perspectives in Ecology and Evolution. New York: Columbia University Press, pp. 302–353, 1984) characterized five types of Sepedophilus larvae based on head structures and the external and internal features of the head of larvae of Sepedophilus type C are described in detail herein. A functional interpretation of structures involved with feeding is made on the basis of morphological and behavioural observations. Types C and D larvae possess tube-like epipharyngeal structures resembling dipteran labial pseudotrachea, which may play an important role in a specialized liquid-feeding process. Based on a preliminary analysis of head characters delimited by Newton (1984) it is shown that mycophagy has evolved once from a predatory ancestor, although some Sepedophilus groups may have mixed feeding strategies. The epipharyngeal tubes are demonstrated to be unique to mycophagous Sepedophilus in Coleoptera whereas the overall head structure is very similar to mycophagous larvae in the family Sphindidae. Pseudotracheen, Larvale Kopfstrukturen und Mycetophagie bei Sepedophilus (Coleoptera: Staphylinidae: Tachyporinae) Newton (in: Wheeler, Q.; Blackwell, M. (eds), Fungus-Insect Relationships: Perspectives in Ecology and Evolution. New York: Columbia University Press, pp. 302–353, 1984) charakterisierte fünf Larventypen von Sepedophilus Gistel, 1856 basierend auf abweichenden Kopfstrukturen. In der vorliegenden Arbeit werden äußere und innere Merkmale des Kopfes von Larven des Typs C detailliert beschrieben. Eine funktionelle Interpretation der am Freßvorhang beteiligten Kopfstrukturen beruht auf morphologischen Befunden und Lebendbeobachtungen. Larven der Typen C und D besitzen röhrenförmige epipharyngeale Strukturen, die an labiale Pseudotracheen von Dipteren erinnern. Sie spielen wahrscheinlich eine wichtige Rolle bei der Ernährung von verflüssigtem Substrat. Eine vorläufige Analyse von Kopfmerkmalen, die von Newton (1984) definiert wurden, ergibt ein nur einmaliges Entstehen von Mycetophagie ausgehend wahrscheinlich von räuberischen Vorfahren. Dabei kann allerdings nicht ausgeschlossen werden, daßSepedophilus -Larven auch unterschiedliche Nahrungsresourcen nutzen können. Die epipharyngealen Röhren kommen außer bei mycetophagen Sepedophilus -Arten bei keiner anderen Coleopterengruppe vor. Die übrigen Kopfstrukturen erinnern dagegen stark an mycetophage Larven der Familie Sphindidae. [source] Sperm defects in mice lacking a functional Niemann,Pick C1 protein,MOLECULAR REPRODUCTION & DEVELOPMENT, Issue 10 2006Jun Fan Abstract The Niemann,Pick C1 (NPC1) gene encodes for a multiple membrane spanning protein, which regulates the trafficking of low-density lipoprotein-mediated endocytosed cholesterol. Mutation of the human NPC1 gene causes Niemann,Pick type C (NPC) disease. The Npc1NIH mice, a model of human NPC disease, bear a spontaneous mutation of the Npc1 gene, and are infertile. In this study, we have performed sperm analysis to search for the cause of male infertility in the Npc1NIH mouse. The number of cauda sperms in Npc1,/, mice was decreased roughly three-and-half-fold of that in wild-type mice. The decreased sperm number in Npc1,/, mice is due, at least in part, to partial arrest of spermatogenesis in the testes, as revealed by histological analysis. Compared to wild-type sperm, Npc1,/, sperm displayed a high frequency of morphological abnormalities, including tailless heads and aberrant heads. In the in vitro fertilization (IVF) assay using cumulus-intact eggs, Npc1,/, sperm failed to produce two-cell embryos. In the IVF assay where zona-free eggs were used, Npc1,/, sperm bound normally but could not fuse with the egg. Further analysis indicated that Npc1,/, sperms are drastically impaired in the binding to the egg zona pellucida, only 14% of the level of wild-type sperm. Moreover, on Npc1,/, cauda sperm, one-third of the total cyritestin protein was not proteolytically processed, while fertilin , was processed normally. Taken together, these results demonstrate that there are multiple defects in sperms from mice lacking a functional NPC1 protein, and these observed sperm defects may result in sterility. Mol. Reprod. Dev. © 2006 Wiley-Liss, Inc. [source] A transient low-frequency quasi-periodic oscillation from the black hole binary GRS 1915+105MONTHLY NOTICES OF THE ROYAL ASTRONOMICAL SOCIETY, Issue 3 2008P. Soleri ABSTRACT We present the results of the timing analysis of five Rossi X-ray Timing Explorer observations of the black hole candidate GRS 1915+105 between 1996 September and 1997 December. The aim was to investigate the possible presence of a type B quasi-periodic oscillation (QPO). Since in other systems this QPO is found to appear during spectral transitions from hard to soft states, we analysed observations characterized by a fast and strong variability, in order to have a large number of transitions. In GRS 1915+105, transitions occur on very short time-scales (,s): to single them out we averaged power density spectra following the regular path covered by the source on a 3D hardness,hardness,intensity diagram. We identified both the type C and the type B QPOs: this is the first detection of a type B QPO in GRS 1915+105. As the spectral transitions have been associated to the emission and collimation of relativistic radio-jets, their presence in the prototypical Galactic jet source strengthens this connection. [source] Motion analysis of a child with Niemann,Pick disease type C treated with miglustatMOVEMENT DISORDERS, Issue 1 2008Alex R. Paciorkowski MD Abstract Niemann,Pick disease type C (NPC) is a progressive neurodegenerative disorder for which there is no effective treatment other than supportive therapy. Recently, the oral medication miglustat has been offered as a possible therapy aimed at reducing pathological substrate accumulation. This article describes the use of computerized three-dimensional motion analysis to evaluate a 3-year-old child with NPC treated with miglustat for 12 months. Motion analysis provided quantitative data on the patient's gait. However, dementia and motor dysfunction progressed despite the treatment, and the patient lost the ability to walk between 9 and 12 months of the study. Motion analysis should be considered among the tools for measuring functional outcomes in future therapeutical trials of patients with neurodegenerative diseases. It is not possible to draw conclusions about miglustat therapy in NPC from a single patient experience. © 2007 Movement Disorder Society [source] Mutations in NPC1 in two Brazilian patients with Niemann-Pick disease type C and progressive supranuclear palsy-like presentationMOVEMENT DISORDERS, Issue 12 2006Clécio Godeiro-Júnior MD [source] Rhythmic cortical myoclonus in Niemann,Pick disease type CMOVEMENT DISORDERS, Issue 9 2006Laura Canafoglia MD Abstract We here describe a patient with late-infantile Niemann,Pick disease type C (NPC) presenting with worsening myoclonus, seizures, cerebellar symptoms, mild mental impairment, and gaze palsy. Electroencephalographic (EEG) ,polymyographic examinations showed abnormally high and diffuse background alpha-activity, enhanced by intermittent photic stimulation. The electromyographic (EMG) showed quasirhythmic myoclonic jerks during motor activation. EEG,EMG frequency analysis (better than jerk-locked back-averaging) demonstrated the cortical origin of the myoclonus. Our observations indicate that cortical myoclonus may occur as the main symptom of NPC. © 2006 Movement Disorder Society [source] Enteritis necroticans ,pigbel' in a Japanese diabetic adultPATHOLOGY INTERNATIONAL, Issue 9 2007Tomomichi Matsuda Enteritis necroticans ,pigbel' is caused by Clostridium perfringens type C but has rarely been reported in developed countries. A 50-year-old Japanese man with untreated diabetes mellitus (DM) presented with diarrhea and abdominal pain. Intraoperative endoscopic and macroscopic examination disclosed segmental annular mucosal lesions characteristic of clostridial enteritis. Clostridial infection type C was verified on pathological, and immunohistochemical analysis. Although rare, the disease is likely to be underdiagnosed. Hence, the pathology and immunohistochemistry of segmental enteritis with annular mucosal lesions should be examined to establish a diagnosis of enteritis necroticans even in mildly affected patients, and especially those with DM. [source] Adrenal rest tumor of the liver: A case report with immunohistochemical investigation of steroidogenesisPATHOLOGY INTERNATIONAL, Issue 3 2000Kazumori Arai Abstract A case of adrenal rest tumor arising in the liver of a 62-year-old male with chronic hepatitis type C is reported. The tumor was clinically non-functioning and required distinction from hepatocellular carcinoma. The yellowish,brown tumor measured 25 × 18 × 15 mm and was located in the subcapsular portion of the right hepatic lobe. Histologically, the tumor presented features similar to those of the adrenal cortex and was predominantly composed of pale cells. Electron micrograph revealed lipid droplets and mitochondria with tubulo,vesicular cristae, consistent with the characteristics of steroid-producing cells. Immunohistochemically, the tumor expressed the adrenal 4 binding protein and a number of enzymes involved in the synthesis of adrenocortical steroids. At surgery, the right adrenal gland was present independently from the liver. This hepatic tumor was considered to be an adrenal rest tumor with steroidogenic capability. [source] Bony ponticles of the atlas (C1) over the groove for the vertebral artery in humans and primates: Polymorphism and evolutionary trendsAMERICAN JOURNAL OF PHYSICAL ANTHROPOLOGY, Issue 1 2004Jean-Marie Le Minor Abstract The aim of this study was to ascertain the distribution in primates of the three possible bony ponticles over the groove for the vertebral artery (ventral, lateral, and dorsal ponticles), in order to attempt to understand the variants observed in humans and to ascertain possible evolutionary trends in primates. The material consisted of 393 atlases of extant nonhuman primates representative of 41 genera, and of 500 human atlases (dried bones of adults). For each atlas, we studied the existence and morphology of the ponticles, and the type of association of these three ponticles on a given side, which are theoretically of eight in number (types A,H). The occurrence of these ponticles varied from complete absence to constant presence, according to the genera and taxa of primates. The presence of each of these ponticles in primates can be interpreted as a primitive or plesiomorphic character, and their absence as a derived or apomorphic character. The strepsirhines-platyrrhines-cercopithecines group, presenting a predominant primitive pattern (type A), appeared to be separated from the colobines-hominoids group, presenting predominant derived patterns (type C in colobines, Pongo pygmaeus, and Pan troglodytes, and the more derived type D in Hylobates, Gorilla gorilla, and Homo sapiens). The last derived stage, corresponding to the disappearance of the three atlantal ponticles (type H), was only observed in some individuals in hominoids. A marked intraspecific polymorphism characterized the hominoids. The presence of lateral and dorsal ponticles in humans appeared to correspond to their persistence within the progressive disappearance of the atlantal ponticles, constituting an evolutionary tendency characteristic of primates and particularly of hominoid evolution. Am J Phys Anthropol, 2004. © 2004 Wiley-Liss, Inc. [source] Ferrocene compounds: methyl 1,-aminoferrocene-1-carboxylateACTA CRYSTALLOGRAPHICA SECTION C, Issue 9 2010Christoph Förster The title compund, [Fe(C5H6N)(C7H7O2)], features one strong intermolecular hydrogen bond of the type N,H...O=C [N...O = 3.028,(2),Å] between the amine group and the carbonyl group of a neighbouring molecule, and vice versa, to form a centrosymmetric dimer. Furthermore, the carbonyl group acts as a double H-atom acceptor in the formation of a second, weaker, hydrogen bond of the type C,H...O=C [C...O = 3.283,(2),Å] with the methyl group of the ester group of a second neighbouring molecule at (x, ,y , , z , ). The methyl group also acts as a weak hydrogen-bond donor, symmetry-related to the latter described C,H...O=C interaction, to a third molecule at (x, ,y , , z + ) to form a two-dimensional network. The cyclopentadienyl rings of the ferrocene unit are parallel to each other within 0.33,(3)° and show an almost eclipsed 1,1,-conformation, with a relative twist angle of 9.32,(12)°. The ester group is twisted slightly [11.33,(8)°] relative to the cylopentadienyl plane due to the above-mentioned intermolecular hydrogen bonds of the carbonyl group. The N atom shows pyramidal coordination geometry, with the sum of the X,N,Y angles being 340,(3)°. [source] Neuropeptide regulators of the juvenile hormone biosynthesis (in vitro) in the beetle, Tenebrio molitor (Coleoptera, Tenebrionidae)ARCHIVES OF INSECT BIOCHEMISTRY AND PHYSIOLOGY (ELECTRONIC), Issue 3 2010Mohatmed Abdel-latief Abstract The genome of Tribolium castaneum encodes two allatostatin [AS type B; W(X)6Wamide and AS type C; PISCF-OH] and one allatotropin (AT) precursor, but no AS type A (FGLamide) (Tribolium Genome Sequencing Consortium, 2008: Nature 452:949,955). Here we studied the activity (in vitro) of peptides derived from these precursors on the synthesis/release of juvenile hormone (JH) III. The corpora cardiaca-corpora allata (CC-CA) complexes of adult females of another tenebrionid beetle, the mealworm Tenebrio molitor, were used. Incubating the gland complexes in a medium containing Trica-AS B3 peptide, we showed that the peptide has allatostatic function in T. molitor. The activity of the type C AS depended on the age of the test animals and their intrinsic rate of JH III biosynthesis. The Trica-AS C peptide inhibited the JH release from CA of 3-day-old females with a high intrinsic rate of JH synthesis, but activated JH release from the CA of 7-day-old females with a lower intrinsic rate of JH production. The allatotropin peptide (Trica-AT) also activated the JH release from the CA of 7-day-old females in a dose-dependent and reversible manner. Unexpectedly, a type A AS derived from the precursor of the American cockroach Periplaneta americana (Peram-AS A2b) inhibited the JH release from the CA of younger and older females in the concentration range of 10,8 to 10,4,M, and the effects were fully reversible in the absence of peptide. These data suggest a complex role of allatoactive neuropeptides in the regulation of JH III biosynthesis in beetles. © 2010 Wiley Periodicals, Inc. [source] Mitotic Epitopes are Incorporated into Age-dependent Neurofibrillary Tangles in Niemann,Pick Disease Type CBRAIN PATHOLOGY, Issue 2 2010Min Zhang MD Abstract The mechanism underlying neurofibrillary tangles (NFTs) in Alzheimer's disease (AD) and other neurodegenerative disorders remains elusive. Niemann,Pick disease type C (NPC) is a kind of genetic neurovisceral disorder in which the intracellular sequestration of cholesterol and other lipids in neurons, NFT formation and neuronal degeneration in brain are the neuropathology hallmarks. The age of onset and progression of the disease vary dramatically. We have analyzed the hippocampus from 17 NPC cases, aged from 7 months to 55 years, to depict the temporal characteristics of NFT formation. Unexpectedly, classic NFT was observed in about 4-year-old NPC brain, suggesting that NFT is not aging dependent, and that juvenile brain neurons satisfy the requirements for NFT formation. NFT in the hippocampus of NPC was significantly increased in number with the advance of age. More importantly, multiple mitotic phase markers, which are not usually found in normal mature neurons, were abundant in the affected neurons and incorporated into NFT. The unusual activation of cdc2/cyclin B kinase and downstream mitotic indices are closely associated with the age-dependent NFT formation, signifying the contribution of abortive cell cycle to neurodegeneration. The cdc2 inhibitors may be therapeutically used for early intervention of neurodegeneration and NFT formation in NPC. [source] 18F-fluorodeoxyglucose and 11C-acetate positron emission tomography are useful modalities for diagnosing the histologic type of thymoma,CANCER, Issue 11 2009Hidekatsu Shibata MD Abstract BACKGROUND: The objective of this study was to clarify the usefulness of positron emission tomography (PET) using18F-fluorodeoxyglucose (FDG) and carbon 11-labeled acetate (AC) for predicting the histologic types and tumor invasiveness of thymoma in a multicenter study. METHODS: Forty thymomas were examined using both FDG-PET and AC-PET before surgery. The histologic types were type A in 1 thymoma, type AB in 12 thymomas, type B1 in 11 thymomas, type B2 in 7 thymomas, type B3 in 6 thymomas, and type C in 3 thymomas. Tumor invasiveness was assessed by pathologic tumor stage and was identified as stage I in 17 tumors, stage II in 17 tumors, stage III in 4 tumors, and stage IV in 2 tumors. FDG and AC uptake was measured as the maximum standard uptake value (SUV). RESULTS: The FDG-SUV in type C thymomas was significantly higher than that in the other types (A-B3; P = .001 , P = .048). The AC-SUV in type A/AB thymomas was significantly higher than that in the other tumor types (B1-C; P < .001 , P = .002). All 3 type C tumors had an FDG-SUV ,6.3, and all 13 type A/AB tumors had an FDG-SUV <6.3 and an AC-SUV ,5.7. All 17 thymomas that had an FDG-SUV <6.3 and an AC-SUV <5.7 were type B1, B2, or B3. Neither the FDG-SUV nor the AC-SUV differed significantly between the stages I/II tumors and stage III/IV tumors. CONCLUSIONS: Although neither the FDG-SUV nor the AC-SUV can predict the invasiveness of thymomas assessed by tumor stage, they are useful for predicting histologic types of thymoma. Thymomas with an FDG-SUV <6.3 and an AC-SUV ,5.7 almost certainly are types A/AB, which is of considerable prognostic and management significance. Cancer 2009. © 2009 American Cancer Society. [source] | |||||||||||||||||||||||||||||||