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L-Ornithine-ketoacid-transaminase deficiency was established in cultured fibroblasts obtained from a patient with hyperornithinaemia (mean ornithine level in serum approximately 100 mumol/l) and gyrate atrophy of the choroid and retina. The deficiency was found both the L-ornithine concentrations of 3.0 mM (about twice the KM value) and 12 mM, indicating that the enzymic defect was not due to a decreased affinity for this substrate. The reliability of the colorimetric assay of ornithine-ketoacid-transaminase activity was established radiochemically. Performance of the radiochemical assay revealed the presence of an impurity in the substrate DL-[2-14C]ornithine - HCl being a strong inhibitor of the enzyme. The passage level and the subcultivation time of the fibroblasts did not influence the enzymic activity.
J M Trijbels, and
R C Sengers, and
J A Bakkeren, and
A F De Kort, and
A F Deutman
March 1978,
American journal of human genetics,
J M Trijbels, and
R C Sengers, and
J A Bakkeren, and
A F De Kort, and
A F Deutman
September 1978,
International ophthalmology,
J M Trijbels, and
R C Sengers, and
J A Bakkeren, and
A F De Kort, and
A F Deutman
November 1977,
The New England journal of medicine,
J M Trijbels, and
R C Sengers, and
J A Bakkeren, and
A F De Kort, and
A F Deutman
January 1982,
Advances in experimental medicine and biology,
J M Trijbels, and
R C Sengers, and
J A Bakkeren, and
A F De Kort, and
A F Deutman
January 1974,
The British journal of ophthalmology,
J M Trijbels, and
R C Sengers, and
J A Bakkeren, and
A F De Kort, and
A F Deutman
December 1988,
American journal of human genetics,
J M Trijbels, and
R C Sengers, and
J A Bakkeren, and
A F De Kort, and
A F Deutman
November 1974,
The British journal of ophthalmology,
J M Trijbels, and
R C Sengers, and
J A Bakkeren, and
A F De Kort, and
A F Deutman
August 1980,
Journal of the Royal Society of Medicine,
J M Trijbels, and
R C Sengers, and
J A Bakkeren, and
A F De Kort, and
A F Deutman
January 1994,
Eye (London, England),
J M Trijbels, and
R C Sengers, and
J A Bakkeren, and
A F De Kort, and
A F Deutman
November 1977,
Biochemical and biophysical research communications,
