PROGRESS IN UNDERSTANDING THE BIOCHEMISTRY OF FRIEDREICHS ATAXIA

被引:2
作者
BARBEAU, A [1 ]
机构
[1] CLIN RES INST MONTREAL,DEPT NEUROBIOL,MONTREAL H2W 1R7,QUEBEC,CANADA
关键词
D O I
10.1016/0166-2236(79)90036-5
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
Recent systematic studies of Friedreich's ataxia, a hereditary degenerative disease, have uncovered a number of previously unsuspected biochemical defects, pieces of a puzzle which may in fact be closely related: a decrease in the activity of lipoamide dehydrogenase in the serum; a decrease in the protein content of high density lipoproteins and in their essential fatty acid composition; a marked decrease in glutamic acid and aspartic acid concentrations in the cerebellum and spinal cord; and a renal loss of taurine and β-alanine. © 1979.
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页码:87 / 90
页数:4
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