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Gyrate atrophy with hyperornithinaemia: different types of responsiveness to vitamin B6.

- nih.gov [PDF] 
S Hayasaka, T Saito, H Nakajima, Y Takaku, … - British Medical Journal, 1981 - bjo.bmj.com
SUMMARY Three cases of Japanese patients with gyrate atrophy of the choroid and
retina with hyperornithinaemia were studied clinically and biochemically. The
types of disease differed in responsiveness to vitamin B6. In-vivo ...
Cited by 33 - Related articles - All 6 versions

A specific enzyme defect in gyrate atrophy.


MI Kaiser-Kupfer, D Valle, LA Del Valle - American journal of ophthalmology, 1978 - ncbi.nlm.nih.gov
To establish the enzyme defect in gyrate atrophy, we measured the activity of
ornithine aminotransferase in phytohemagglutinin stimulated lymphocytes in a
patient with gyrate atrophy, her daughter, and three normal controls. The ...
Cited by 27 - Related articles - All 2 versions

Clinical trial of vitamin B6 for gyrate atrophy of the choroid and retina.


RG Weleber, NG Kennaway - Ophthalmology, 1981 - ncbi.nlm.nih.gov
Seven patients with gyrate atrophy and deficiency of
ornithine-delta-aminotransferase were studied for in vivo pyridoxine
responsiveness; three responded to oral vitamin B6 with over 50% reduction ...
Cited by 31 - Related articles - All 3 versions

Ocular findings in patients with gyrate atrophy on pyridoxine and low-protein, low-arginine …


EL Berson, VE Shih, PL Sullivan - Ophthalmology, 1981 - ncbi.nlm.nih.gov
Five patients, ages 12 to 30, with gyrate atrophy have shown substantial (60% or
greater) decreases in plasma ornithine concentrations within four to eight weeks
when placed on a therapeutic trial of low-protein (10-15 g/day), ...
Cited by 19 - Related articles - All 3 versions

Ornithine ketoacid aminotransferase in the bovine eye.

- iovs.org [PDF] 
S Hayasaka, T Shiono, Y Takaku, K Mizuno - Investigative ophthalmology & visual science, 1980 - ncbi.nlm.nih.gov
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 ...
Cited by 22 - Related articles - All 2 versions

Clinical and biochemical heterogeneity in gyrate atrophy.


MI Kaiser-Kupfer, D Valle, AJ Bron - American journal of ophthalmology, 1980 - ncbi.nlm.nih.gov
Two patients had milder variants of the usual form of gyrate atrophy. Although
the appearance of the chorioretinal degeneration was identical, the progression
of the clinical signs and symptoms were slower with night blindness, ...
Cited by 16 - Related articles

Gyrate atrophy of the choroid and retina. Biochemical considerations and experience with an …


D Valle, M Walser, S Brusilow, MI Kaiser- … - Ophthalmology, 1981 - ncbi.nlm.nih.gov
Ornithine-delta-aminotransferase deficiency is the primary biochemical defect in
gyrate atrophy of the choroid and retina and results in the characteristic
accumulation of ornithine. An additional consequence of this inborn error ...
Cited by 16 - Related articles - All 3 versions

Gyrate atrophy of the choroid and retina: characterization of mutant ornithine …

- nih.gov [PDF] 
NG Kennaway, L Stankova, MK Wirtz, RG … - American journal of human genetics, 1989 - pubmedcentral.nih.gov
Page 1. Am. J. Hum. Genet. 44:344-352, 1989 Gyrate Atrophy of the Choroid and
Retina: Characterization of Mutant Ornithine Aminotransferase ...
Cited by 10 - Related articles - All 5 versions

Clinical trials of vitamin B6 and proline supplementation for gyrate atrophy of the choroid and …

- nih.gov [PDF] 
S Hayasaka, T Saito, H Nakajima, O … - British Medical Journal, 1985 - bjo.bmj.com
Page 1. doi:10.1136/bjo.69.4.283 1985;69;283-290 Br. J. Ophthalmol. and K. Tada
S. Hayasaka, T. Saito, H. Nakajima, O. Takahashi, K. Mizuno ...
Cited by 12 - Related articles - All 7 versions

Ornithine ketoacid transaminase deficiency in gyrate atrophy of the choroid and retina.

- nih.gov [PDF] 
VE Shih, EL Berson, R Mandell, SY Schmidt - American Journal of Human Genetics, 1978 - pubmedcentral.nih.gov
INTRODUCTION Gyrate atrophy of the choroid and retina is a chorioretinal
degeneration with an autosomal recessive mode of inheritance [1-3]. Patients
report night blindness and loss of peripheral vision between the ages of 10 ...
Cited by 59 - Related articles - All 3 versions


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