Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P00790 (PGA)
 2,475 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Continuous injection of L-pyroglutamate (L-PGA) into the rat striatum induces a lesion with three regions: a necrotic core, a rim of pyknotic cells, and a peripheral spongiose region. The L-PGA was administered through an implanted intrastriatal cannula coupled to an Alzet osmotic pump loaded with one of three doses of L-PGA (3, 5, or 13 times the normal amount of L-PGA/g wet wt rat forebrain (23 nmol/g). The magnitude of the lesion was dependent upon the concentration of buffered L-PGA and the duration of continuous pumping. The necrotic region contained macrophages and neutrophils, while condensed neurons and oligodendroglial cells were present in the pyknotic region. The spongiose region contained vacuolated neuropil and degenerating nerve cells and oligodendroglia. The spongiose region blends with the normal neuropil. A population of aspiny neurons were identified throughout the spongiose region. These neurons stained positive for NADPH diaphorase and demonstrated a somatostatin-like immunoreactivity similar to that of the aspiny neurons spared in Huntington's disease and in the neurotoxin-induced striatal-lesioned rat models of Huntington's disease.
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PMID:Chronic intrastriatal L-pyroglutamate: neuropathology and neuron sparing like Huntington's disease. 256 47

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.
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PMID:Striatal deficiency of L-pyroglutamic acid in Huntington's disease is accompanied by increased plasma levels. 297 22

Intrastriatal injections of L-Pyroglutamate (L-PGA) in mice produced behavioral and neuropathological effects that resemble in part the kainate-injected rat striatal model of Huntington's Disease (HD). The behavioral responses induced after unilateral injections of L-PGA included circling, postural asymmetry of head and trunk and possible dyskinesias. The neuropil in the injected striatum contained dilated profiles, degenerating neurons and oligodendroglia, and numerous phagocytic microglial-like cells. A dose response relation existed. The size of the lesion (expressed as a percent volume of the striatum destroyed) ranged from 1 +/- 0.18% at 0.02 mumoles to 20.2 +/- 3.97% at 200 mumoles L-PGA (pH = 7.3). L-PGA is a weak neurotoxin when compared to kainic acid. Several factors raise interest in the possible role of L-PGA in HD, including the recently reported elevated plasma levels of L-PGA in some HD patients, and these considered in the discussion.
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PMID:L-pyroglutamate: an alternate neurotoxin for a rodent model of Huntington's disease. 623 48