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LIPID DEFICIENCY IN THE CENTRAL NERVOUS SYSTEM OF LANDRACE PIGLETS AFFECTED WITH CONGENITAL TREMOR AIII, A FORM OF CEREBROSPINAL HYPOMYELINOGENESIS
Author(s) -
Patterson D. S. P.,
Sweasey D.,
Harding J. D. J.
Publication year - 1972
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/j.1471-4159.1972.tb03816.x
Subject(s) - spinal cord , cerebellum , white matter , cerebrum , central nervous system , biology , cerebrospinal fluid , endocrinology , grey matter , medicine , cord , cerebroside , myelin , anatomy , biochemistry , neuroscience , magnetic resonance imaging , surgery , radiology
—Congenital tremor, type AIII, is characterized by partial agenesis of the white matter of the CNS affecting mainly the spinal cord. The percentage water content of the fresh cord is consistently higher than normal and other parts of the CNS are sporadically affected. The total lipid content (mg/g fresh tissue) is markedly decreased in the cord but brain stem and cerebellum are less severely deficient; the cerebrum is barely deficient. Total amounts of cholesterol, cerebroside and phospholipid (mmol/part) are significantly reduced predominantly in cerebellum, brain stem and cord. Total DNA and protein contents are decreased to a significant extent only in spinal cord. Broadly similar lipid changes are found in fixed tissues. The data are consistent with sub‐normal myelination, associated with a deficiency of oligodendrocytes.