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Pathway Function (pathway + function)

Distribution by Scientific Domains

Medical Sciences	57%
Life Sciences	42%

Selected Abstracts

Atrioventricular Nodal Reentrant Tachycardia in Children: Effect of Slow Pathway Ablation on Fast Pathway Function

JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 3 2002
GEORGE F. VAN HARE M.D.
AV Nodal Reentry in Children.Introduction: Prior studies in adults have shown significant shortening of the fast pathway effective refractory period after successful slow pathway ablation. As differences between adults and children exist in other characteristics of AV nodal reentrant tachycardia (AVNRT), we sought to characterize the effect of slow pathway ablation or modification in a multicenter study of pediatric patients. Methods and Results: Data from procedures in pediatric patients were gathered retrospectively from five institutions. Entry criteria were age < 21 years, typical AVNRT inducible with/without isoproterenol infusion, and attempted slow pathway ablation or modification. Dual AV nodal pathways were defined as those with > 50 msec jump in A2-H2 with a 10-msec decrease in A1-A2. Successful ablation was defined as elimination of AVNRT inducibility. A total of 159 patients (age 4.4 to 21 years, mean 13.1) were studied and had attempted slow pathway ablation. AVNRT was inducible in the baseline state in 74 (47%) of 159 patients and with isoproterenol in the remainder. Dual AV nodal pathways were noted in 98 (62%) of 159 patients in the baseline state. Ablation was successful in 154 (97%) of 159 patients. In patients with dual AV nodal pathways and successful slow pathway ablation, the mean fast pathway effective refractory period was 343 ± 68 msec before ablation and 263 ± 64 msec after ablation. Mean decrease in the fast pathway effective refractory period was 81 ± 82 msec (P < 0.0001) and was not explained by changes in autonomic tone, as measured by changes in sinus cycle length during the ablation procedure. Electrophysiologic measurements were correlated with age. Fast pathway effective refractory period was related to age both before (P = 0.0044) and after ablation (P < 0.0001). AV block cycle length was related to age both before (P = 0.0005) and after ablation (P < 0.0001). However, in dual AV nodal pathway patients, the magnitude of change in the fast pathway effective refractory period after ablation was not related to age. Conclusion: Lack of clear dual AV node physiology is common in pediatric patients with inducible AVNRT (38%). Fast pathway effective refractory period shortens substantially in response to slow pathway ablation. The magnitude of change is large compared with adult reports and is not completely explained by changes in autonomic tone. Prospective studies in children using autonomic blockade are needed. [source]

Gli3 null mice display glandular overgrowth of the developing stomach

DEVELOPMENTAL DYNAMICS, Issue 4 2005
Jae H. Kim
Abstract The role of the Hedgehog signaling pathway in various aspects of gut development is still poorly understood. In the developing stomach, Sonic (Shh) and Indian (Ihh) hedgehog are expressed in both distinct and overlapping regions. Loss of Sonic hedgehog function in the stomach results in a glandular phenotype of intestinal transformation and overgrowth. These changes are reminiscent of the pre-malignant lesion, intestinal metaplasia. To determine the role of Hedgehog-related transcription factors, Gli2 and Gli3, in Shh signaling during stomach development, we conducted a mutant analysis of glandular stomach from Shh, Gli2, and Gli3 mutant mice. Although Gli2 principally mediates the activator function of Shh, surprisingly we observed minimal changes in glandular development in the Gli2 mutant stomach. Furthermore, Gli3, which typically functions as a repressor of Hedgehog signal, showed a striking phenocopy of the glandular expansion and intestinal transformation found in Shh mutant stomach. A reduction in apoptotic events was seen in all mutant stomachs with no appreciable changes in proliferation. Both Shh and Gli3 mutant stomachs displayed early changes of intestinal transformation but these did not impact on the overall differentiation of the gastric epithelium. Interestingly, the observation that Gli3 shares a similar glandular phenotype to Shh mutant stomach reveals a possible novel role of Gli3 activator in the developing stomach. The embryonic stomach is a unique model of the Hedgehog pathway function and one that may help to uncover some of the mechanisms underlying the development of intestinal metaplasia. Developmental Dynamics 234:984,991, 2005. © 2005 Wiley-Liss, Inc. [source]

Genetic variation for dorsal,ventral patterning of the Drosophila melanogaster eggshell

EVOLUTION AND DEVELOPMENT, Issue 2 2005
Lisa M. Goering
Summary Patterning of the insect eggshell is an excellent system for exploring the molecular basis of phenotypic variation. In Drosophila melanogaster, two dorsal,anterior respiratory appendages are produced in response to signaling through the Epidermal growth factor receptor (Egfr). Previous work implicates Egfr pathway function in both intraspecific variation for dorsal appendage spacing (DAS) on the eggshell, as well as interspecific differences in dorsal appendage number and location. To test the hypothesis that genetic variation in Egfr contributes to variation in eggshell patterning, we have made use of naturally occurring intraspecific variation for DAS as a model quantitative trait. We found that there is substantial segregating genetic variation for DAS in D. melanogaster, and have tested for associations with 289 common polymorphisms in the Egfr locus. A marginal association was seen with two polymorphic sites in Egfr; however, we failed to replicate these findings in a second population, or in a modified quantitative complementation test designed to specifically test the effects of the putative polymorphisms. Therefore, we conclude that the polymorphisms we have identified in Egfr do not contribute to variation in DAS, and further work is required to understand the genetic architecture of this trait. [source]

Bile duct proliferation in liver-specific Jag1 conditional knockout mice: Effects of gene dosage,

HEPATOLOGY, Issue 2 2007
Kathleen M. Loomes
The Notch signaling pathway is involved in determination of cell fate and control of cell proliferation in multiple organ systems. Jag1 encodes a ligand in the Notch pathway and has been identified as the disease-causing gene for the developmental disorder Alagille syndrome. Evidence from the study of human disease and mouse models has implicated Jag1 as having an important role in the development of bile ducts. We have derived a conditional knockout allele (Jag1loxP) to study the role of Jag1 and Notch signaling in liver and bile duct development. We crossed Jag1loxP mice with a transgenic line carrying Cre recombinase under the control of the albumin promoter and ,-fetoprotein enhancer to ablate Jag1 in hepatoblasts. The liver-specific Jag1 conditional knockout mice showed normal bile duct development. To further decrease Notch pathway function, we crossed the Jag1 conditional knockout mice with mice carrying the hypomorphic Notch2 allele, and bile duct anatomy remained normal. When Jag1 conditional mice were crossed with mice carrying the Jag1 null allele, the adult progeny exhibited striking bile duct proliferation. Conclusion: These results indicate that Notch signaling in the liver is sensitive to Jag1 gene dosage and suggest a role for the Notch pathway in postnatal growth and morphogenesis of bile ducts. (HEPATOLOGY 2007.) [source]

Afferent pathway dysfunction in children with primary nocturnal enuresis

INTERNATIONAL JOURNAL OF UROLOGY, Issue 2 2010
Linya Lv
Objectives: To investigate afferent pathway dysfunction in children with primary nocturnal enuresis by measuring pudendal somatosensory evoked potential and tibial somatosensory evoked potential. Methods: Subjects with primary nocturnal enuresis, 36 boys and 18 girls, aged from 5 to 16 years, were enrolled in this study: 24 subjects had complicated primary enuresis (CPE) and 30 subjects had monosymptomatic primary enuresis (MPE). There were no differences in bodyweight or gender between the MPE and CPE groups (P > 0.05). All of the children underwent physical examination, urine analysis, urinary ultrasound and spinal magnetic resonance imaging. Only subjects without urological and neurological abnormalities (with the exception of spina bifida occulta, which was found in some of the patients) were included in this neurophysiological study. Results: There were 20 children who were positively recorded with pudendal somatosensory evoked potential in the CPE group, and all of the children in the MPE group were positively recorded (P < 0.05). Positive records of tibial somatosensory evoked potential were successfully achieved in both groups. Furthermore, the pudendal and tibial conductive velocity were slower as compared to the normal range, especially in children in the CPE group (P < 0.001). Conclusions: Afferent pathway function may be impaired by some factors, which should be considered by both clinicians and parents. [source]

Dissociation between cutaneous silent period and laser evoked potentials in assessing neuropathic pain

MUSCLE AND NERVE, Issue 3 2009
A. Truini MD
Abstract In this study we investigate whether the cutaneous silent period (CSP),an inhibitory response evoked in hand muscles by painful digital nerve stimulation,is useful for assessing nociceptive pathway function in patients with neuropathic pain. In 40 patients with peripheral neuropathy (21 without and 19 with neuropathic pain) we recorded the CSP in the abductor digiti minimi after fifth digit stimulation and also recorded laser evoked potentials (LEPs) after stimulation applied to the ulnar territory of the hand. Although the LEP amplitude was significantly lower in patients with pain than in those without (P < 0.005), the CSP duration did not differ between groups (P > 0.50). Pain intensity correlated significantly with LEP amplitudes (P < 0.005) but not with CSP duration (P > 0.5). Our findings indicate that the CSP is not useful for assessing nociceptive pathway function in patients with neuropathic pain. Muscle Nerve, 2008 [source]

Tamo selectively modulates nuclear import in Drosophila

GENES TO CELLS, Issue 4 2003
Svetlana Minakhina
Background: The NF-,B/Rel pathway functions in the establishment of dorsal-ventral polarity and in the innate humoral and cellular immune response in Drosophila. An important aspect of all NF-,B/Rel pathways is the translocation of the Rel proteins from the cytoplasm to the nucleus, where they function as transcription factors. Results: We have identified a new protein, Tamo, which binds to Drosophila Rel protein Dorsal, but not to Dorsal lacking the nuclear localization sequence. Tamo does not bind to the other Drosophila Rel proteins, Dif and Relish. The Tamo-Dorsal complex forms in the cytoplasm and Tamo also interacts with a cytoplasmically orientated nucleoporin. In addition Tamo binds the Ras family small GTPase, Ran. Tamo functions during oogenesis and, based on phenotypic analysis, controls the levels of nuclear Dorsal in early embryos. It further regulates the accumulation of Dorsal in the nucleus after immune challenge. Conclusions:Tamo has an essential function during oogenesis. Tamo interacts with Dorsal and proteins that are part of the nuclear import machinery. We propose that tamo modulates the levels of import of Dorsal and other proteins. [source]