Mitochondrial ornithine aminotransferase (OAT) catalyzes the reversible reaction of ornithine and alpha-ketoglutarate to form glutamate semialdehyde and glutamate (Ohura et al. 1982). The active enzyme is a hexamer (Shen et al. 1998). Inherited OAT deficiency leads to ornithine accumulation in vivo and gyrate atrophy of the choroid and retina (Brody et al. 1992; Valle and Simell 2001).
Katunuma, N, Ohura, T, Kominami, E, Tada, K
Beaudet, AL, Scriver, CR, Sly, WS, Valle, D
Schirmer, T, Hohenester, E, Jansonius, JN, Shen, BW, Hennig, M
Obie, C, Valle, D, Kaiser-Kupfer, M, Robert, MF, Sipila, I, Mitchell, GA, Steel, G, Michaud, J, Fontaine, G, Brody, LC
ornithine-oxo-acid transaminase activity of OAT hexamer [mitochondrial matrix]
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