Hsp27-2D-gel electrophoresis is a diagnostic tool to differentiate primary desminopathies from myofibrillar myopathies.
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.
Department of Neurology, Medical Faculty, University of Bonn, Sigmund Freud Str. 25, 53127 Bonn, FRG.
Christoph S
CS
Clemen
Dirk
D
Fischer
Udo
U
Roth
Stéphanie
S
Simon
Patrick
P
Vicart
Kanefusa
K
Kato
Anna M
AM
Kaminska
Matthias
M
Vorgerd
Lev G
LG
Goldfarb
Bruno
B
Eymard
Norma B
NB
Romero
Bertrand
B
Goudeau
Thomas
T
Eggermann
Klaus
K
Zerres
Angelika A
AA
Noegel
Rolf
R
Schröder
D000595:Amino Acid Sequence; D003893:Desmin / Q000032:analysis; D003937:Diagnosis, Differential; D015180:Electrophoresis, Gel, Two-Dimensional; D055551:HSP27 Heat-Shock Proteins; D006360:Heat-Shock Proteins / Q000032:analysis* / Q000378:metabolism; D006801:Humans; D006863:Hydrogen-Ion Concentration; D018832:Molecular Chaperones; D008969:Molecular Sequence Data; D018482:Muscle, Skeletal / Q000737:chemistry / Q000473:pathology; D020914:Myopathies, Structural, Congenital / Q000175:diagnosis*; D009363:Neoplasm Proteins / Q000032:analysis* / Q000378:metabolism; D010766:Phosphorylation; D020033:Protein Isoforms / Q000032:analysis / Q000378:metabolism; D038203:alpha-Crystallin B Chain / Q000032:analysis / Q000378:metabolism
D003893:Desmin; D055551:HSP27 Heat-Shock Proteins; C057725:HSPB1 protein, human; D006360:Heat-Shock Proteins; D018832:Molecular Chaperones; D009363:Neoplasm Proteins; D020033:Protein Isoforms; D038203:alpha-Crystallin B Chain