Erythrocyte membrane abnormalities in patients with amyotrophic chorea with acanthocythosis. Part 2. Abnormal degradation of membrane proteins.
Bands 2.1, 3 and 4.2 of the erythrocyte membrane proteins in chorea-acanthocytosis are self-digested faster than those in normal controls. The junction between spectrin and band 3 protein at the cytoplasmic side of the patients' erythrocyte membranes may have some conformational defects. The conformational defects of the proteins may be responsible for the low fluidity in the interior of the patients' erythrocyte membranes. These basic molecular defects may be widespread in other extraneural cell membranes as well as in neuronal cell membranes.
Asano
K
K
Osawa
Y
Y
Yanagisawa
N
N
Takahashi
Y
Y
Oshima
M
M
D001798:Blood Proteins / Q000378:metabolism*; D002819:Chorea / Q000097:blood / Q000235:genetics*; D004910:Erythrocyte Membrane / Q000378:metabolism*; D006801:Humans; D006816:Huntington Disease / Q000097:blood; D008560:Membrane Fluidity; D008565:Membrane Proteins / Q000378:metabolism*; D009133:Muscular Atrophy / Q000097:blood / Q000235:genetics*; D013577:Syndrome
D001798:Blood Proteins; D008565:Membrane Proteins