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guanidinoacetic acid/atrophy

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Gyrate atrophy of the choroid and retina with hyperornithinemia. Deficient formation of guanidinoacetic acid from arginine.

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Patients with gyrate atrophy of the choroid and retina have 10- to 20-fold increased ornithine concentrations in body fluids and significantly reduced activity of ornithine aminotransferase in lymphocytes and cultured fibroblasts. We administered intravenous arginine to six patients and six controls

Creatine corrects muscle 31P spectrum in gyrate atrophy with hyperornithinaemia.

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BACKGROUND Eye fundus destruction and type II muscle fiber atrophy in gyrate atrophy of the choroid and retina with hyperornithinaemia (GA) may be mediated by elevated ornithine concentrations which strongly inhibit creatine biosynthesis. This results in deficiency of creatine phosphate (PCr), a key
Guanidinoacetic acid (GAA) has been shown to spare arginine (ARG) requirements in chickens. ARG plays a critical role in enhancing growth and preventing right ventricular hypertrophy (RVH) in broiler chickens subjected to hypobaric hypoxia. However, ARG is not available as a feed grade supplement in
High levels of guanidinoacetate acid (GAA) deteriorate growth response in broiler chickens. We propose using coenzyme Q10 , an antioxidant, and taurine (TAU), a methyl donor, to cope with the situation when high level of GAA included in diet. GAA was supplemented at 0 (control), 0.75, 1.5 and 2.25

Significance of guanidino compounds in non-dialyzed patients with chronic renal failure.

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Various guanidino compounds were determined in 48 non-dialyzed patients with chronic renal failure. The patients were divided into two groups, as follows: group A, chronic glomerulonephritis and polycystic kidney; and group B, diabetes nephropathy, lupus nephritis and renal amyloidosis. Six kinds of

Inborn errors of creatine metabolism and epilepsy.

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Creatine metabolism disorders include guanidinoacetate methyltransferase (GAMT) deficiency, arginine:glycine amidinotransferase (AGAT) deficiency, and the creatine transporter (CT1-encoded by SLC6A8 gene) deficiency. Epilepsy is one of the main symptoms in GAMT and CT1 deficiency, whereas the
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