Gyrate Atrophy of the Choroid and Retina (GA) : Toxic Effects of Ornithine and Long-Term Therapy with an Arginine-Restricted DietGyrate atrophyOrnithineNight blind-nessGA is characterized by progressive chorioretinal degeneration and hyperornithinemia and is due to an inherited deficiency of ornithine...
Myopathy with tubular aggregates and gyrate atrophy of the choroid and retina due to hyperornithinaemia. J Neurol Neurosurg Psychiatry. 2007;78(6):656–7. Epub 2006 Nov 6.CrossRefPubMedPubMedCentral 3. Kaiser-Kupfer MI, Caruso RC, Valle D, Reed GF. Use of an arginine-restricted diet ...
Summary Westudied thehumanornithine aminotransferase (OAT) gene,mRNA,andenzyme activity infibroblasts froma family with gyrateatrophy (G.A.) ofthechoroid andretina, using a normal humanOATcDNAas a probe. Thefamily consists ofan affected patient, whoisheterozygous fora partial deletion ofthefunctional...
17. Use of an arginine-restricted diet to slow progression of visual loss in patients with gyrate atrophy 精氨酸限制饮食减缓视网膜及脉络膜脑回样萎缩患者的视力丧失 18. Research and Development of Limiter for Flxed and Gyrate Crane 固定回转式起重机力矩限制器的研究与开发 ...
Low Protein Intake in the Management of Gyrate Atrophy of the Choroid and Retina with Hyperornithinemia: 12 Years of Follow-upeye diseases, protein intake, case studies, girls, amino acid metabolism, diet therapyThe objective and electroretinographic modifications that occurred during a 12-year ...
Gyrate Atrophy of the Choroid and Retina (GACR) currently lacks any effective treatment and a highly restricted, yet cumbersome, low-arginine diet is the only available therapeutic option. In our work, we found that replacement of ornithine-aminotransferase (OAT) activity in the liver lowers ...
DIETtext>Hyperornithinemia associated with gyrate atrophy of the choroid and retina is a rare, autosomal recessive disorder resulting from a deficiency of the mitochondrial matrix enzyme, ornithine d-aminotransferase (OAT). This enzyme catalyses the pyridoxal phosphate-dependent transamination of ornithine...
DietTime FactorsNo abstract available.doi:10.1136/bjo.58.1.3TakkiKBritish Journal of OphthalmologyK. Takki, Gyrate atrophy of the choroid and retina associated with hyperornithinaemia, Br. J. Ophthalmol. 58 (1974) 3-23.Takki K. Gyrate atrophy of the choroid and retina associated with hyper...
Fluorescein angiography showed hyperfluorescence in the area of gyrate atrophy. Her plasma ornithine level and plasma tiramine level were 1 90 U mol/l and 357 U mol/l. respectively. A rigid schedule of low protein diet including near total elimination of arginine with supplementation of essential...
Patients are treated with an arginine-restricted diet, to limit ornithine load, or the administration of Vitamin B6, a precursor of the OAT coenzyme pyridoxal phosphate. Although the clinical and genetic aspects of GA are known for many years, the enzymatic phenotype of pathogenic variants and ...