Hsp27-2D-gel electrophoresis is a diagnostic tool to differentiate primary desminopathies from myofibrillar myopathies.

Abstract	Small heat shock proteins prevent abnormal protein folding and accumulation. We analyzed the expression of hsp27 and alphaB-crystallin in skeletal muscle specimens of patients with desminopathies, plectinopathies, myotilinopathy, and other myofibrillar myopathies by means of differential centrifugation, 2D-gel electrophoresis, Western blotting, and mass spectrometry. Hsp27-P82 and -P15 as well as alphaB-crystallin-P59 and -P45 are the major serine phosphorylation isoforms in normal and diseased human skeletal muscle. 2D-gel-electrophoresis revealed spots of hsp27 in a range of pH 5.3-6.4 in samples of all skeletal muscle specimens, except for the seven desminopathies. They indicated a shift of the main hsp27-spot to alkaline pH degrees, which may help to differentiate primary desminopathies from other myopathies with structural pathology of the desmin cytoskeleton.
Authors	Christoph S Clemen, Dirk Fischer, Udo Roth, Stéphanie Simon, Patrick Vicart, Kanefusa Kato, Anna M Kaminska, Matthias Vorgerd, Lev G Goldfarb, Bruno Eymard, Norma B Romero, Bertrand Goudeau, Thomas Eggermann, Klaus Zerres, Angelika A Noegel, Rolf Schröder
Journal	FEBS letters (FEBS Lett) Vol. 579 Issue 17 Pg. 3777-82 (Jul 04 2005) ISSN: 0014-5793 [Print] England
PMID	15978589 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References	Desmin HSP27 Heat-Shock Proteins HSPB1 protein, human Heat-Shock Proteins Molecular Chaperones Neoplasm Proteins Protein Isoforms alpha-Crystallin B Chain
Topics	Amino Acid Sequence Desmin (analysis) Diagnosis, Differential Electrophoresis, Gel, Two-Dimensional HSP27 Heat-Shock Proteins Heat-Shock Proteins (analysis, metabolism) Humans Hydrogen-Ion Concentration Molecular Chaperones Molecular Sequence Data Muscle, Skeletal (chemistry, pathology) Myopathies, Structural, Congenital (diagnosis) Neoplasm Proteins (analysis, metabolism) Phosphorylation Protein Isoforms (analysis, metabolism) alpha-Crystallin B Chain (analysis, metabolism)

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