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Selective Pattern (selective + pattern)

Distribution by Scientific Domains

Medical Sciences	50%

Selected Abstracts

A BAC transgenic mouse model to analyze the function of astroglial SPARCL1 (SC1) in the central nervous system

GLIA, Issue 9 2008
Jill M. Weimer
Abstract Extracellular matrix associated Sparc-like 1 (SC1/SPARCL1) can influence the function of astroglial cells in the developing and mature central nervous system (CNS). To examine SC1's significance in the CNS, we generated a BAC transgenic mouse model in which Sc1 is expressed in radial glia and their astrocyte derivatives using the astroglial-specific Blbp (Brain-lipid binding protein; [Feng et al., (1994) Neuron 12:895,908]) regulatory elements. Characterization of these Blbf-Sc1 transgenic mice show elevated Sc1 transcript and protein in an astroglial selective pattern throughout the CNS. This model provides a novel in vivo system for evaluating the role of SC1 in brain development and function, in general, and for understanding SC1's significance in the fate and function of astroglial cells, in particular. © 2008 Wiley-Liss, Inc. [source]

Hippocampal Pathology in the Human Neuronal Ceroid-Lipofuscinoses: Distinct Patterns of Storage Deposition, Neurodegeneration and Glial Activation

BRAIN PATHOLOGY, Issue 4 2004
Jaana Tyynelä
The neuronal ceroid-lipofuscinoses (NCLs) are recessively inherited lysosomal storage diseases, currently classified into 8 forms (CLN1-CLN8). Collectively, the NCLs constitute the most common group of progressive encephalopathies of childhood, and present with visual impairment, psychomotor deterioration and severe seizures. Despite recent identification of the underlying disease genes, the mechanisms leading to neurodegeneration and epilepsy in the NCLs remain poorly understood. To investigate these events, we examined the patterns of storage deposition, neurodegeneration, and glial activation in the hippocampus of patients with CLN1, CLN2, CLN3, CLN5 and CLN8 using histochemistry and immunohistochemistry. These different forms of NCL shared distinct patterns of neuronal degeneration in the hippocampus, with heavy involvement of sectors CA2-CA4 but relative sparing of CA1. This selective pattern of degeneration was also observed in immunohistochemically identified interneurons, which exhibited a graded severity of loss according to phenotype, with calretinin-positive interneurons relatively spared. Furthermore, glial activation was also regionally specific, with microglial activation most pronounced in areas of greatest neuronal loss, and astrocyte activation prominent in areas where neuronal loss was less evident. In conclusion, the NCLs share a common pattern of selective hippocampal pathology, distinct from that seen in the majority of temporal lobe epilepsies. [source]

Widespread Policy Disruption: Terrorism, Public Risks, and Homeland Security

POLICY STUDIES JOURNAL, Issue 2 2009
Peter J. May
We address theoretical and empirical aspects of policy disruptions that affect multiple areas of policymaking. Our theorizing leads us to consider the effects of widespread disruptions in gaining the attention of elected officials, in affecting policymaking, and in reshaping the involvement of federal agencies. Our empirical analyses concern the threat of terrorism in the United States and its implications for public risk subsystems over the past 25 years. Our analyses of the attention of policymakers and resultant policymaking volatility show selective patterns of subsystem disruption related to the threat of terrorism. We show that capturing the attention of policymakers in multiple subsystems is insufficient to motivate heightened levels of policymaking across the board. In addition, we find more muted impacts for federal agency involvement than might have been expected from the massive reorganization that created the Department of Homeland Security. More generally, the disjunctions we observe show the powerful influence of policy subsystems in buffering against widespread policy disruptions. [source]

Muscle magnetic resonance imaging involvement in muscular dystrophies with rigidity of the spine

ANNALS OF NEUROLOGY, Issue 2 2010
Eugenio Mercuri MD
Objective The aim of the study was to evaluate whether the visual analysis of muscle magnetic resonance imaging scans can identify specific patterns of muscle involvement. Methods We assessed scans from 83 patients with muscle disorders characterized by rigidity of the spine secondary to mutations in 4 different genes. The conditions studied were rigid spine syndrome (SEPN1 defects), Bethlem myopathy, and Ullrich congenital muscular dystrophy, allelic disorders caused by Col6A1, Col6A2, and Col6A3 mutations, the autosomal dominant form of Emery,Dreifuss muscular dystrophy (LMNA defects) and calpain-deficient limb girdle muscular dystrophy (CAPN3 defects). The scans of 25 patients affected by other myopathies were also reviewed as a control group. The scans were compared with the previously described patterns. Results In 82% of the scans in the study group (68/83) the patterns were classified as "typical" of 1 of the 5 forms studied, and in 7 (8%) were consistent with 1 of the reported patterns but not entirely typical. With one exception, the patterns identified were always consistent with the appropriate genetic diagnosis. The remaining scans (9%) had only minimal changes and were uninformative. None of the scans of the 25 patients in the control group had patterns that could be classified as typical of the 5 forms examined. The sensitivity to detect selective patterns in relation to the genetic diagnosis was 0.9. Interpretation These findings suggest that muscle magnetic resonance imaging could be used in clinical practice as an additional tool in the differential diagnosis of muscle disorders with prominent spinal rigidity. ANN NEUROL 2010;67:201,208 [source]