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Speculations on the nature of the metabolic defects in Tay‐Sachs, Niemann‐Pick, Gaucher舗s and Alzheimer舗s diseases, and metachromatic leucodystrophy
Author(s) -
Rouser George,
Kritchevsky Gene,
Galli Claudio
Publication year - 1965
Publication title -
journal of the american oil chemists' society
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.512
H-Index - 117
eISSN - 1558-9331
pISSN - 0003-021X
DOI - 10.1007/bf02635578
Subject(s) - metachromatic leukodystrophy , glucocerebroside , tay sachs disease , niemann–pick disease , sphingomyelin , disease , enzyme , gaucher's disease , sphingolipid , ceramide , medicine , biochemistry , biology , glucocerebrosidase , cholesterol , apoptosis
It is concluded that the defect in Tay‐Sachs disease is for an enzyme degrading a monosialoganglioside, in Niemann‐Pick disease for an enzyme degrading sphingomyelin or possibly ceramide, in chronic Gaucher舗s disease for an enzyme degrading a glucocerebroside, and in metachromatic leucodystrophy for an enzyme for degradation of sulfatide. Alzheimer舗s disease does not appear to involve any specific changes in lipid composition. An hypothesis to explain the findings in Alzheimer舗s disease is presented.