|1.||Wasant, Pornswan: 1 article (07/2012)|
|2.||Liammongkolkul, Somporn: 1 article (07/2012)|
|3.||Vatanavicharn, Nithiwat: 1 article (07/2012)|
|4.||Ratanarak, Pisanu: 1 article (07/2012)|
|5.||Sathienkijkanchai, Achara: 1 article (07/2012)|
|6.||Bangal, Surekha: 1 article (01/2012)|
|7.||Gogri, Pratik: 1 article (01/2012)|
|8.||Dhaytadak, Priyanka: 1 article (01/2012)|
|9.||Bhandari, Akshay: 1 article (01/2012)|
|10.||Chabli, A: 1 article (06/2009)|
09/01/2000 - "In human hereditary OAT deficiency the elevated intraocular concentrations of Orn are considered to be a cause of gyrate atrophy. "
02/01/1991 - "Molecular pathology of gyrate atrophy of the choroid and retina due to ornithine aminotransferase deficiency."
06/01/1988 - "Molecular basis of ornithine aminotransferase deficiency in B-6-responsive and -nonresponsive forms of gyrate atrophy."
04/01/1984 - "A lack of substrate OAT specificity indicates OAT deficiency such as occur in gyrate atrophy of the choroid and retina could impair metabolism of ketoacids. "
03/01/1978 - "We conclude that OKT deficiency is closely associated with the genetic defect in gyrate atrophy of the choroid and retina and that genetic heterogeneity exists in this disease."
02/01/1983 - "Gyrate atrophy of the retina and choroid is an autosomal recessive disease characterized by progressive retinal degeneration and ornithine aminotransferase deficiency. "
02/01/2006 - "Ornithine delta-aminotransferase (OAT) deficiency (McKusick 258870) is associated with hyperornithinaemia, thought to be the cause of the progressive retinal degeneration that occurs in this disorder. "
12/05/1989 - "Little is known about OAT modulation in retinal tissue, although chorio-retinal degeneration is the predominant feature in a hereditary disorder of OAT deficiency, gyrate atrophy. "
02/01/2000 - "To determine whether chronic, systemic reduction of ornithine can prevent this form of retinal degeneration, we used an arginine-restricted diet to maintain long term reduction of ornithine in a mouse model of OAT-deficiency (Oat(-/-)) produced by gene targeting. "
|3.||Muscular Diseases (Myopathy)
07/11/2012 - "Fifty-eight patients were diagnosed with amino acid disorders, including 20 cases (34.5%) with maple syrup urine disease, 13 (22.4%) with phenylketonuria and hyperphenylalaninemia, 13 (22.4%) with nonketotic hyperglycinemia, 9 (15.5%) with urea cycle defects, 2 (3.4%) with classical homocystinuria, and 1 (1.7%) with ornithine aminotransferase deficiency. "
|2.||Ornithine-Oxo-Acid Transaminase (Ornithine Aminotransferase)