Nestin Is Not Essential for Development of the CNS But Required for Dispersion of Acetylcholine Receptor Clusters at the Area of Neuromuscular Junctions

Abstract Nestin is expressed in many different progenitors during development including those of the CNS, heart, skeletal muscle, and kidney. The adult expression is mainly restricted to the subependymal zone and dentate gyrus of the brain, the neuromuscular junction, and renal podocytes. In addition, this intermediate filament protein has served as a marker of neural stem/progenitor cells for close to … Continue reading

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A comparative clinical, pathological, biochemical and genetic study of fused in sarcoma proteinopathies.

T Lashley, JD Rohrer, R Bandopadhyay, C Fry, Z Ahmed, AM Isaacs, JH Brelstaff, B Borroni, JD Warren, C Troakes, A King, S Al-Saraj, J Newcombe, N Quinn, K Ostergaard, HD Schroder, M Bojsen-Moller, H Braendgaard, NC Fox, MN Rossor, AJ Lees, JL Holton, and T Revesz Medline Abstract Neuronal intermediate filament inclusion disease and atypical frontotemporal lobar degeneration are … Continue reading

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Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments.

V Swarup, D Phaneuf, C Bareil, J Robertson, GA Rouleau, J Kriz, and JP Julien Medline Abstract Transactive response DNA-binding protein 43 ubiquitinated inclusions are a hallmark of amyotrophic lateral sclerosis and of frontotemporal lobar degeneration with ubiquitin-positive inclusions. Yet, mutations in TARDBP, the gene encoding these inclusions are associated with only 3% of sporadic and familial amyotrophic lateral sclerosis. … Continue reading

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Muscle creatine kinase deficiency triggers both actin depolymerization and desmin disorganization by advanced glycation end-products in dilated cardiomyopathy

Cell Biology Intermediate Filaments Abstract Alterations in the balance of cytoskeleton as well as energetic proteins are involved in the cardiac remodeling occurring in dilated cardiomyopathy (DCM). We used 2D-DIGE proteomics as a discovery approach to identify key molecular changes taking place in a temporally controlled model of DCM triggered by cardiomyocyte-specific Serum Response Factor KO in mice. We identified … Continue reading

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