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BOVINE GENERALIZED GLYCOGENOSIS
Author(s) -
RICHARDS R. B.,
EDWARDS J. R.,
COOK R. D.,
WHITE R. R.
Publication year - 1977
Publication title -
neuropathology and applied neurobiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.538
H-Index - 95
eISSN - 1365-2990
pISSN - 0305-1846
DOI - 10.1111/j.1365-2990.1977.tb00568.x
Subject(s) - glycogen , glycogen storage disease , skeletal muscle , electron microscope , enzyme , biology , pathology , pathological , endocrinology , biochemistry , medicine , physics , optics
The clinical, pathological, and biochemical features of a glycogen storage disease in beef cattle are described. The light and electron microscope changes in skeletal muscle, heart and the central nervous system were similar to the lesions that have been described in glycogenosis Type II, or Pompe's disease, in man. The stored intracytoplasmic material was shown histochemically and electron microscopically to be glycogen. Biochemical assay of liver, skeletal muscle, and heart demonstrated a deficiency of the lysosomal enzyme α‐ 1 , 4‐glucosidase.