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Lysosomal abnormalities in cultured schwann cells from a patient with peripheral neuropathy and continuous muscle fiber activity
Author(s) -
Askanas Valerie,
Engel W. King,
Berginer Vladimir M.,
Odenwald Ward F.,
Galdi Albert
Publication year - 1981
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410100307
Subject(s) - schwann cell , sural nerve , vacuole , acid phosphatase , lysosome , pathology , peripheral neuropathy , microbiology and biotechnology , medicine , biology , chemistry , endocrinology , enzyme , biochemistry , cytoplasm , diabetes mellitus
Schwann cell cultures were established from a sural nerve containing large membrane‐bound vaculoes in its schwann cells, obtained from a patient with neuropathy and continuous muscle fiber activity. cultured Schwann cell contained may large membrane‐bound vaculoles, presumably lysosomes, resembling those present in the biopsied nerve. the acid phosphatase reaction was excessive in the patient's cultured schwann cells but practically negative in normal cultured Schwann cells. this study Indicates that the patient's neuropathy is primary dysschwannian with abnormal lysosomes as a major abnormality.