منابع مشابه
Oligomeric State and Thermal Stability of Apo- and Holo- Human Ornithine δ-Aminotransferase
Human ornithine δ-aminotransferase (hOAT) (EC 2.6.1.13) is a mitochondrial pyridoxal 5'-phosphate (PLP)-dependent aminotransferase whose deficit is associated with gyrate atrophy, a rare autosomal recessive disorder causing progressive blindness and chorioretinal degeneration. Here, both the apo- and holo-form of recombinant hOAT were characterized by means of spectroscopic, kinetic, chromatogr...
متن کاملOrnithine ketoacid aminotransferase in the bovine eye.
Ornithine ketoacid aminotransferase in bovine ocular tissues was studied biochemically. The retinal pigment epithelium, ciliary body, iris, and neuroretina showed high specific activity. The cornea and choroid revealed a lower activity. Aqueous humor, lens, vitreous body, and sclera showed no activity. The pH optima of the enzyme in the retinal pigment epithelium and ciliary body were near 8.0.
متن کاملGyrate Atrophy of the Choroid and Retina Diagnosed by Ornithine-δ-aminotransferase Gene Analysis: A Case Report
A pair of 19-year-old female identical twins was referred to our hospital with progressive visual loss. They exhibited bilateral chorioretinal atrophy involving the midperiphery on fundoscopy and fluorescein angiography. Bilateral visual field constriction was noted on dynamic Goldmann perimetry, and a markedly impaired response was observed on both photopic and scotopic electroretinograms. Cys...
متن کاملExpression defect of ornithine aminotransferase gene in gyrate atrophy.
A generalized deficiency in the mitochondrial enzyme, ornithine aminotransferase (OAT: EC 2.6.1.13), is the hallmark of gyrate atrophy (GA), a hereditary degenerative disease of the choroid and retina of the eye that leads to blindness. A human OAT cDNA, previously constructed and characterized in our laboratory, and anti-human OAT antibody were used as probes to examine the OAT gene, mRNA and ...
متن کاملImmunocytochemical localization of ornithine aminotransferase in rat ocular tissues.
Gyrate atrophy of the choroid and retina is a rare inherited form of chorioretinal degeneration due to a deficiency of ornithine aminotransferase (OAT). We localized the enzyme in rat ocular tissues using immunocytochemical procedures. Immunoreactivity was observed in the epithelia of ciliary body, iris, and lens. Retinal pigment epithelium and Müller cells were immunoreactive in the retina. A ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Plant Signaling & Behavior
سال: 2008
ISSN: 1559-2324
DOI: 10.4161/psb.6771