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Inborn errors of GABA metabolism
Author(s) -
Gibson Kenneth M.,
Nyhan William L.,
Jaeken Jaak
Publication year - 1986
Publication title -
bioessays
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.175
H-Index - 184
eISSN - 1521-1878
pISSN - 0265-9247
DOI - 10.1002/bies.950040107
Subject(s) - glutamate decarboxylase , metabolism , aminobutyric acid , enzyme , biochemistry , glutamate dehydrogenase , gamma aminobutyric acid , biology , chemistry , glutamate receptor , receptor
Defects in man in four steps of 4‐aminobutyric acid (GABA) metabolism may interefere with the function of this major inhibitory neurotransmitter. Glutamic acid decarboxylase, 4‐aminobutyric acid aminotransferase, succinic semialdehyde dehydrogenase, and homocarnosinase are closely identified with the brain, but two of these enzymes are expressed in cultured peripheral cells, which may permit novel approaches to the study of the metabolism and regulation of GABA.