Striatal deficiency of L-pyroglutamic acid in Huntington's disease is accompanied by increased plasma levels.
L-Pyroglutamic acid (L-PGA), a cyclized glutamate analogue, was measured in plasma, cerebrospinal fluid and brain tissue of patients with Huntington's disease (HD) and controls. In HD, plasma L-PGA was elevated and possibly reflects an increased requirement of cell membranes to be protected against peroxidative damage. L-PGA was decreased in caudate and putamen of HD patients. We suggest that striatal deficiency of L-PGA in HD is a consequence of neuronal loss which characteristically occurs in HD striatum.
Uhlhaas
S
S
Institute of Human Genetics, University of Bonn, F.R.G.
Lange
H
H
D000328:Adult; D000367:Age Factors; D000368:Aged; D003342:Corpus Striatum / Q000378:metabolism*; D006801:Humans; D006816:Huntington Disease / Q000097:blood / Q000378:metabolism* / Q000503:physiopathology; D008875:Middle Aged; D011760:Pyrrolidinones / Q000378:metabolism*; D011761:Pyrrolidonecarboxylic Acid / Q000097:blood / Q000134:cerebrospinal fluid / Q000378:metabolism*; D013378:Substantia Nigra / Q000378:metabolism
D011760:Pyrrolidinones; D011761:Pyrrolidonecarboxylic Acid