David Valle

Professor

1971 …2020

Research output per year

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Research Output

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1982

Carnitine deficiency presenting as familial cardiomyopathy: A treatable defect in carnitine transport

Waber, L. J., Valle, D., Neill, C., DiMauro, S. & Shug, A., Nov 1982, In : The Journal of pediatrics. 101, 5, p. 700-705 6 p.

Research output: Contribution to journalArticle

Cataplexy in variant forms of Niemann‐Pick disease

Kandt, R. S., Emerson, R. G., Singer, H. S., Valle, D. L. & Moser, H. W., Sep 1982, In : Annals of neurology. 12, 3, p. 284-288 5 p.

Research output: Contribution to journalArticle

Mannosidosis in an adult

Montgomery, T. R., Thomas, G. H. & Valle, D. L., Dec 1 1982, In : Johns Hopkins Medical Journal. 151, 3, p. 113-116 4 p.

Research output: Contribution to journalArticle

Mutant cell lines resistant to azetidine-2-carboxylic acid: Changes in the biosynthesis of proline from glutamic acid

Smith, R. J., Lodato, R. F. & Valle, D., Jan 1 1982, In : Federation Proceedings. 41, 4, p. No. 5236

Research output: Contribution to journalArticle

Screening for lethal genetic disease.

Brusilow, S. W., Valle, D. L., Batshaw, M. L. & Waber, L. J., Oct 1 1982, In : Pediatrics. 70, 4, p. 647-648 2 p.

Research output: Contribution to journalArticle

Treatment of alanine induced hyperammonemia with benzoate or phenylacetate in lysinuric protein intolerance (LPI)

Simell, O., Rajantie, J. & Valle, D., Jan 1 1982, In : Pediatric research. 16, 8, p. No. 40

Research output: Contribution to journalArticle

1981

Depakene (VPA) induced hyperammonemia (HA)

Batshaw, M. L., Valle, D. & Brusilow, S. W., Jan 1 1981, In : Pediatric research. 15, 4 II, p. No. 1559

Research output: Contribution to journalArticle

Effects of arginine-free meals on ureagenesis in cats

Stewart, P. M., Batshaw, M., Valle, D. & Walser, M., Jan 1 1981, In : American Journal of Physiology - Endocrinology and Metabolism. 4, 4, p. 310-315 6 p.

Research output: Contribution to journalArticle

Gyrate Atrophy of the Choroid and Retina: Biochemical Considerations and Experience with an Arginine-Restricted Diet

Valle, D., Walser, M., Brusilow, S., Kaiser-Kupfer, M. I. & Takki, K., 1981, In : Ophthalmology. 88, 4, p. 325-330 6 p.

Research output: Contribution to journalArticle

Gyrate atrophy of the choroid and retina in a cat

Valle, D. L., Boison, A. P., Jezyk, P. & Aguirre, G., Jan 1 1981, In : Investigative Ophthalmology and Visual Science. 20, 2, p. 251-255 5 p.

Research output: Contribution to journalArticle

Mutant cell lines resistant to azetidine carboxylic acid: Quantitative and qualitative differences in pyrroline-5-carboxylate synthase activity

Smith, R. J., Lodato, R. F., Valle, D. L. & Kazakis, A., Apr 15 1981, In : Biochemical and Biophysical Research Communications. 99, 3, p. 789-795 7 p.

Research output: Contribution to journalArticle

Partial trisomy 6q, due to balanced maternal translocation (6;22) (q21;p13) or (q21;pter)

Stamberg, J., Shapiro, J., Valle, D., Kuhajda, F. P., Thomas, G. H. & Wissow, L., 1981, In : Unknown Journal. 19, 2, p. 122-125 4 p.

Research output: Contribution to journalArticle

Regulation of proline biosynthesis: The inhibition of pyrroline-5-carboxylate synthase activity by ornithine

Lodato, R. F., Smith, R. J., Valle, D., Phang, J. M. & Aoki, T. T., Sep 1981, In : Metabolism. 30, 9, p. 908-913 6 p.

Research output: Contribution to journalArticle

Unsuccessful treatment of phenylketonuria with tyrosine

Batshaw, M. L., Valle, D. & Bessman, S. P., Jul 1981, In : The Journal of pediatrics. 99, 1, p. 159-160 2 p.

Research output: Contribution to journalArticle

Visual Results of a Long-Term Trial of a Low-Arginine Diet in Gyrate Atrophy of Choroid and Retina

Kaiser-Kupfer, M. I., de Monasterio, F., Valle, D., Walser, M. & Brusilow, S., Jan 1 1981, In : Ophthalmology. 88, 4, p. 307-310 4 p.

Research output: Contribution to journalArticle

1980

Clinical and biochemical heterogeneity in gyrate atrophy

Kaiser-Kupfer, M. I., Valle, D. & Bron, A. J., Feb 1980, In : American journal of ophthalmology. 89, 2, p. 219-222 4 p.

Research output: Contribution to journalArticle

Cultured muscle fibers of gyrate atrophy (GA) patients: Tubules, ornithine toxicity, and 1-ornithine-2-oxoacid aminotransferase (OAT) deficiency

Askanas, V., Valle, D. & Kaiser-Kupfer, M. I., Jan 1 1980, In : Neurology. 30, 4, p. NMD4

Research output: Contribution to journalArticle

Gyrate atrophy of the choroid and retina: Improved visual function following reduction of plasma ornithine by diet

Kaiser-Kupfer, M. I., De Monasterio, F. M., Valle, D., Walser, M. & Brusilow, S., Jan 1 1980, In : Science. 210, 4474, p. 1128-1131 4 p.

Research output: Contribution to journalArticle

Gyrate atrophy of the choroid and retina. Amino acid metabolism and correction of hyperornithinemia with an arginine-deficient diet

Valle, D., Walser, M., Brusilow, S. W. & Kaiser-Kupfer, M., 1980, In : Journal of Clinical Investigation. 65, 2, p. 371-378 8 p.

Research output: Contribution to journalArticle

Open Access

Homocystinuria due to cystathionine β-synthase deficiency: Clinical manifestations and therapy

Valle, D., Pai, G. S., Thomas, G. H. & Pyeritz, R. E., Jan 1 1980, In : Johns Hopkins Medical Journal. 146, 3, p. 110-117 8 p.

Research output: Contribution to journalArticle

Increased sensitivity of gyrate atrophy (GA) fibroblasts and cultured muscle cells to ornithine (ORN) toxicity

Valle, D., Askanas, V. & Kupfer, M. K., Jan 1 1980, In : Pediatric research. 14, 4 II, p. No. 616

Research output: Contribution to journalArticle

Taurine (TAU) transport in cultured human fibroblasts from normals and patients with retinitis pigmentosa (RP)

Tanaka, Y., Valle, D. L. & Maumenee, I. H., Jan 1 1980, In : Investigative Ophthalmology and Visual Science. 19, Suppl. 4, 1 p.

Research output: Contribution to journalArticle

1979

Genetic evidence for a common enzyme catalyzing the second step in the degradation of proline and hydroxyproline.

Valle, D., Goodman, S. I., Harris, S. C. & Phang, J. M., Nov 1979, In : The Journal of clinical investigation. 64, 5, p. 1365-1370 6 p.

Research output: Contribution to journalArticle

Open Access

Increased sensitivity of gyrate atrophy fibroblasts to ornithine toxicity

Valle, D., Boison, A. P. & Kaiser-Kupfer, M. I., Jan 1 1979, In : Pediatric research. 13, 4 II, p. No. 606

Research output: Contribution to journalArticle

Syndromes due to chromosomal abnormalities: Partial trisomy 22, interstitial deletion of the long arm of 13, and trisomy 8

Pai, G. S., Thomas, G. H., Leonard, C. O., Ward, J. C., Valle, D. L. & Pyeritz, R. E., Dec 1 1979, In : Johns Hopkins Medical Journal. 145, 4, p. 162-169 8 p.

Research output: Contribution to journalArticle

1978

A specific enzyme defect in gyrate atrophy

Kaiser-Kupfer, M. I., Valle, D. & Del Valle, L. A., Jan 1 1978, In : American journal of ophthalmology. 85, 2, p. 200-204 5 p.

Research output: Contribution to journalArticle

1977

Gyrate atrophy of the choroid and retina: Deficiency of ornithine aminotransferase in transformed lymphocytes

Valle, D., Kaiser Kupfer, M. I. & Del Valle, L. A., Jan 1 1977, In : Proceedings of the National Academy of Sciences of the United States of America. 74, 11, p. 5159-5161 3 p.

Research output: Contribution to journalArticle

Open Access

Proline oxidase in cultured mammalian cells

Downing, S. J., Phang, J. M., Kowaloff, E. M., Valle, D. & Smith, R. J., Jun 1977, In : Journal of Cellular Physiology. 91, 3, p. 369-376 8 p.

Research output: Contribution to journalArticle

The stiff skin syndrome: new genetic and biochemical investigations

Singer, H., Valle, D., Rogers, J. & Thomas, G., Jan 1 1977, In : Birth Defects: Original Article Series. 13, 3 B, p. 254-255 2 p.

Research output: Contribution to journalArticle

1976

Type II hyperprolinemia. Δ1 Pyrroline 5 carboxylic acid dehydrogenase deficiency in cultured skin fibroblasts and circulating lymphocytes

Valle, D., Goodman, S. I., Applegarth, D. A., Shih, V. E. & Phang, J. M., Jan 1 1976, In : Unknown Journal. 58, 3, p. 598-603 6 p.

Research output: Contribution to journalArticle

Open Access
1975

A radioisotopic assay for proline oxidase activity

Phang, J. M., Downing, S. J., Valle, D. L. & Kowaloff, E. M., Feb 1975, In : The Journal of laboratory and clinical medicine. 85, 2, p. 312-317 6 p.

Research output: Contribution to journalArticle

Increased sensitivity of lymphocyte Δ1pyrroline-5- carboxylate reductase to inhibition by proline with transformation

Valle, D., Blaese, R. M. & Phang, J. M., Dec 1 1975, In : Nature. 253, 5488, p. 214-216 3 p.

Research output: Contribution to journalArticle

Proline biosynthesis and degradation in mammalian cells and tissue

Phang, J. M., Valle, D. & Kowaloff, E. M., Dec 1 1975, In : Annals of Clinical and Laboratory Science. 5, 4, p. 298-302 5 p.

Research output: Contribution to journalArticle

Puromycin effect on amino acid transport: differential rates of carrier protein turnover

Phang, J. M., Valle, D. L., Fisher, L. & Granger, A., Jan 1 1975, In : American Journal of Physiology. 228, 1, p. 23-26 4 p.

Research output: Contribution to journalArticle

1974

Type 2 hyperprolinemia: Absence of Δ1-pyrroline-5- carboxylic acid dehydrogenase activity

Valle, D. L., Phang, J. M. & Goodman, S. I., Jan 1 1974, In : Science. 185, 4156, p. 1053-1054 2 p.

Research output: Contribution to journalArticle

1973

A radioisotopic assay for ornithine-δ-transaminase

Phang, J. M., Downing, S. J. & Valle, D., Sep 1973, In : Analytical biochemistry. 55, 1, p. 272-277 6 p.

Research output: Contribution to journalArticle

A radioisotopic assay for Δ1-pyrroline-5-carboxylate reductase

Phang, J. M., Downing, S. J. & Valle, D., Sep 1973, In : Analytical biochemistry. 55, 1, p. 266-271 6 p.

Research output: Contribution to journalArticle

Proline biosynthesis: Multiple defects in Chinese hamster ovary cells

Valle, D., Downing, S. J., Harris, S. C. & Phang, J. M., Aug 21 1973, In : Biochemical and Biophysical Research Communications. 53, 4, p. 1130-1136 7 p.

Research output: Contribution to journalArticle

Proline inhibition of pyrroline-5-carboxylate reductase: Differences in enzymes obtained from animal and tissue culture sources

Valle, D., Downing, S. J. & Phang, J. M., Oct 15 1973, In : Biochemical and Biophysical Research Communications. 54, 4, p. 1418-1424 7 p.

Research output: Contribution to journalArticle

1971

Compartmental Analysis of Sulfobromophthalein Transport in Normal Patients and Patients with Hepatic Dysfunction

Quarfordt, S. H., Hilderman, H. L., Valle, D. & Waddell, E., 1971, In : Gastroenterology. 60, 2, p. 246-255 10 p.

Research output: Contribution to journalArticle