TY - JOUR
T1 - Lipid abnormalities in hereditary neuropathy. Part 1. Serum non-polar lipids
AU - Yao, Jeffrey K.
AU - Ellefson, Ralph D.
AU - Dyck, Peter James
N1 - Funding Information:
The clinical, genetic, nerve conduction, electromyographic (EMG), and pathologic characteristics of inherited motor and sensory neuropathies are being studied extensively and are providing information on the natural history, the nosology, and the nature of nerve fiber degeneration. For most varieties of these inherited neuropathies, and especially for the more common ones, a biochemical abnormality has not been found (Dyck and Lambert 1968; Thomas, Calne and King 1972; Dyck 1975). Abnormalities of lipid metabolism have been demonstrated in Refsum's This investigation was supported in part by Contract AM2-2200; Grants NSO-7541 from N1H; MDA 12 Center grant, and the Upton Fund.
PY - 1976/10
Y1 - 1976/10
N2 - The non-polar lipids from sera of 54 patients, with various types of hereditary motor and sensory neuropathies, and from 72 healthy subjects were evaluated. A small but highly significant decrease in the percentage of linoleate to total fatty acids in both cholesteryl ester and triglyceride fractions was found in the sera of the neuropathy patients, except in those who had dominantly inherited sensory neuropathy (HSN-I) and who had spinocerebellar degeneration with retinitis pigmentosa and other features (SpC+). A significant decrease of serum lecithin-cholesterol acyltransferase activity was also found in those patients with hereditary motor and sensory neuropathies, Type I and Type II (two types of peroneal muscular atrophy). The biochemical basis of these abnormalities is not apparent. The biochemical abnormalities reported here have been found in several neurologic disorders and hence are unlikely to be disease-specific.
AB - The non-polar lipids from sera of 54 patients, with various types of hereditary motor and sensory neuropathies, and from 72 healthy subjects were evaluated. A small but highly significant decrease in the percentage of linoleate to total fatty acids in both cholesteryl ester and triglyceride fractions was found in the sera of the neuropathy patients, except in those who had dominantly inherited sensory neuropathy (HSN-I) and who had spinocerebellar degeneration with retinitis pigmentosa and other features (SpC+). A significant decrease of serum lecithin-cholesterol acyltransferase activity was also found in those patients with hereditary motor and sensory neuropathies, Type I and Type II (two types of peroneal muscular atrophy). The biochemical basis of these abnormalities is not apparent. The biochemical abnormalities reported here have been found in several neurologic disorders and hence are unlikely to be disease-specific.
UR - http://www.scopus.com/inward/record.url?scp=0017172233&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0017172233&partnerID=8YFLogxK
U2 - 10.1016/0022-510X(76)90168-4
DO - 10.1016/0022-510X(76)90168-4
M3 - Article
C2 - 185334
AN - SCOPUS:0017172233
SN - 0022-510X
VL - 29
SP - 161
EP - 175
JO - Journal of the neurological sciences
JF - Journal of the neurological sciences
IS - 2-4
ER -