David Valle

Professor

1971 …2019
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Research Output 1971 2019

1971

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

Quarfordt, S. H., Hilderman, H. L., Valle, D. & Waddell, E., Jan 1 1971, In : Gastroenterology. 60, 2, p. 246-255 10 p.

Research output: Contribution to journalArticle

Sulfobromophthalein
Liver
Secretory Rate
Intravenous Injections
Hepatitis
1973

A radioisotopic assay for ornithine-δ-transaminase

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

Research output: Contribution to journalArticle

Ornithine-Oxo-Acid Transaminase
Ornithine
Enzyme activity
Chromatography
Cations

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

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

Research output: Contribution to journalArticle

Pyrroline Carboxylate Reductases
Assays
Oxidoreductases
Column chromatography
Proline

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

Biosynthesis
Cricetulus
Proline
Ovary
Cells

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

Pyrroline Carboxylate Reductases
Tissue culture
Proline
Oxidoreductases
Animals
1974

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

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

Research output: Contribution to journalArticle

Carboxylic Acids
Oxidoreductases
Fibroblasts
Hyperprolinemia type 2
pyrroline
1975

A radioisotopic assay for proline oxidase activity

Phang, J. M., Downing, S. J., Valle, D. & Kowaloff, E. M., 1975, In : The Journal of Laboratory and Clinical Medicine. 85, 2, p. 312-317 6 p.

Research output: Contribution to journalArticle

Proline Oxidase
Assays
Ion Exchange Chromatography
Radioactivity
Chromatography

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

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

Research output: Contribution to journalArticle

Proline
Oxidoreductases
Lymphocytes
Enzymes
Amino Acids

Proline biosynthesis and degradation in mammalian cells and tissue

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

Research output: Contribution to journalArticle

Biosynthesis
Proline
Assays
Cells
Tissue

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

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

Research output: Chapter in Book/Report/Conference proceedingChapter

Puromycin
Carrier Proteins
Aminoisobutyric Acids
Amino Acids
Half-Life
1976

Type II hyperprolinemia. Δ 1 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., 1976, In : Journal of Clinical Investigation. 58, 3, p. 598-603 6 p.

Research output: Contribution to journalArticle

Carboxylic Acids
Oxidoreductases
Fibroblasts
Lymphocytes
Skin
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., 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

Gyrate Atrophy
Ornithine-Oxo-Acid Transaminase
Lymphocytes
Phytohemagglutinins
Enzymes

Proline oxidase in cultured mammalian cells

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

Research output: Contribution to journalArticle

Proline Oxidase
Cultured Cells
Cells
Proline
Enzymes

The stiff skin syndrome: new genetic and biochemical investigations

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

Research output: Contribution to journalArticle

Molecular Biology
Skin
Mobility Limitation
Proteoglycans
Glycosaminoglycans
1978

A specific enzyme defect in gyrate atrophy

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

Research output: Contribution to journalArticle

Gyrate Atrophy
Ornithine-Oxo-Acid Transaminase
Nuclear Family
Enzymes
Phytohemagglutinins

CLUSTER OF TRISOMY 13 LIVE BIRTHS

Pai, G. S., Valle, D., Thomas, G. & Rosenbaum, K., Mar 18 1978, In : The Lancet. 311, 8064, p. 613 1 p.

Research output: Contribution to journalArticle

Space-Time Clustering
Chromosomes, Human, 13-15
Trisomy
Live Birth
Newborn Infant
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 : Journal of Clinical Investigation. 64, 5, p. 1365-1370 6 p.

Research output: Contribution to journalArticle

Hydroxyproline
Proline
1-Pyrroline-5-Carboxylate Dehydrogenase
Oxidoreductases
Enzymes

Increased sensitivity of gyrate atrophy fibroblasts to ornithine toxicity

Valle, D., Boison, A. P. & Kaiser-Kupfer, M. I., 1979, In : Pediatric Research. 13, 4 II

Research output: Contribution to journalArticle

Gyrate Atrophy
Ornithine
Fibroblasts

NEW PATHWAYS OF NITROGEN EXCRETION IN INBORN ERRORS OF UREA SYNTHESIS

Brusilow, S., Valle, D. & Batshaw, M., Sep 1 1979, In : The Lancet. 314, 8140, p. 452-454 3 p.

Research output: Contribution to journalArticle

Urea
Nitrogen
Glutamine
Argininosuccinic Acid
Inborn Errors Metabolism

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. & Pyeritz, R. E., 1979, Johns Hopkins Medical Journal. 4 ed. Vol. 145. p. 162-169 8 p.

Research output: Chapter in Book/Report/Conference proceedingChapter

Chromosomes, Human, 21-22 and Y
Chromosomes, Human, 6-12 and X
Chromosome Disorders
Growth Disorders
Multiple Abnormalities
1980

Clinical and biochemical heterogeneity in gyrate atrophy

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

Research output: Contribution to journalArticle

Gyrate Atrophy
Vitamin B 6
Ornithine
Night Blindness
Genetic Heterogeneity
Gyrate Atrophy
Keto Acids
Ornithine
Transaminases
Muscles

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., 1980, In : Science. 210, 4474, p. 1128-1131 4 p.

Research output: Contribution to journalArticle

Gyrate Atrophy
Ornithine
Diet
Arginine
Dark Adaptation

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

Gyrate Atrophy
Arginine
Ornithine
Diet
Amino Acids

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

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

Research output: Chapter in Book/Report/Conference proceedingChapter

Cystathionine
Homocystinuria
Pyridoxine
Early Diagnosis
Neonatal Screening

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

Valle, D., Askanas, V. & Kupfer, M. K., 1980, In : Pediatric Research. 14, 4 II

Research output: Contribution to journalArticle

Gyrate Atrophy
Ornithine
Muscle Cells
Cultured Cells
Fibroblasts

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

Tanaka, Y., Valle, D. & Maumenee, I. H., 1980, In : Investigative Ophthalmology and Visual Science. 19, Suppl. 4, p. 185 1 p.

Research output: Contribution to journalArticle

Retinitis Pigmentosa
Taurine
Fibroblasts
1981

Depakene (VPA) induced hyperammonemia (HA)

Batshaw, M. L., Valle, D. & Brusilow, S. W., 1981, In : Pediatric Research. 15, 4 II

Research output: Contribution to journalArticle

Hyperammonemia
Valproic Acid

Effects of arginine-free meals on ureagenesis in cats.

Stewart, P. M., Batshaw, M., Valle, D. & Walser, M., Oct 1981, In : The American journal of physiology. 241, 4

Research output: Contribution to journalArticle

Ornithine
Ornithine-Oxo-Acid Transaminase
Hyperammonemia
Meals
Arginine

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
Ornithine
Arginine
Diet
Ornithine-Oxo-Acid Transaminase

Gyrate atrophy of the choroid and retina in a cat

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

Research output: Contribution to journalArticle

Gyrate Atrophy
Ornithine-Oxo-Acid Transaminase
Ornithine
Felidae
Atrophy

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. & Kazakis, A., Apr 15 1981, In : Biochemical and Biophysical Research Communications. 99, 3, p. 789-795 7 p.

Research output: Contribution to journalArticle

Ornithine-Oxo-Acid Transaminase
Carboxylic Acids
Proline
Cells
Cell Line

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. & Wissow, L. S., 1981, In : Clinical Genetics. 19, 2, p. 122-125 4 p.

Research output: Contribution to journalArticle

Mothers
Imperforate Anus
Encephalocele
Disorders of Sex Development
Umbilical Hernia

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., 1981, In : Metabolism. 30, 9, p. 908-913 6 p.

Research output: Contribution to journalArticle

Ornithine-Oxo-Acid Transaminase
Ornithine
Proline
Glutamic Acid
Gyrate Atrophy

Unsuccessful treatment of phenylketonuria with tyrosine

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

Research output: Contribution to journalArticle

Fortified Food
Phenylketonurias
Intelligence Tests
Child Development
Neurologic Manifestations

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., 1981, In : Ophthalmology. 88, 4, p. 307-310 4 p.

Research output: Contribution to journalArticle

Gyrate Atrophy
Ornithine
Arginine
Diet
Dark Adaptation
1982

Acute Hydrocephalus in Hurler’s Syndrome

Shinnar, S., Singer, H. & Valle, D., 1982, In : American Journal of Diseases of Children. 136, 6, p. 556-557 2 p.

Research output: Contribution to journalArticle

Cerebrospinal Fluid Shunts
Mucopolysaccharidosis I
X Ray Computed Tomography
Acute Disease
Preschool Children

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

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

Research output: Contribution to journalArticle

Carnitine
Cardiomyopathies
Heart Diseases
Reference Values
Kidney

Cataplexy in variant forms of Niemann-Pick disease

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

Research output: Contribution to journalArticle

Niemann-Pick Diseases
Cataplexy
Protriptyline
Narcolepsy
Telemetry

Gyrate atrophy of the choroid and retina.

Valle, D. & Kaiser-Kupfer, M., 1982, In : Progress in Clinical and Biological Research. 82, p. 123-134 12 p.

Research output: Contribution to journalArticle

Uveal Diseases
Gyrate Atrophy
Ornithine-Oxo-Acid Transaminase
Recessive Genes
Pyridoxine

Mannosidosis in an adult

Montgomery, T. R., Thomas, G. H. & Valle, D., 1982, Johns Hopkins Medical Journal. 3 ed. Vol. 151. p. 113-116 4 p.

Research output: Chapter in Book/Report/Conference proceedingChapter

Mannosidase Deficiency Diseases
Mannosidases
Mucopolysaccharidosis I
Mannose
Biochemistry

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., 1982, In : Federation proceedings. 41, 4

Research output: Contribution to journalArticle

Azetidinecarboxylic Acid
Proline
Glutamic Acid
Cell Line

Screening for lethal genetic disease.

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

Research output: Contribution to journalArticle

Inborn Errors Amino Acid Metabolism
Inborn Genetic Diseases
Genetic Testing
Newborn Infant

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

Simell, O., Rajantie, J. & Valle, D., 1982, In : Pediatric Research. 16, 8

Research output: Contribution to journalArticle

Hyperammonemia
Benzoates
Alanine
Lysinuric Protein Intolerance
phenylacetic acid
1983

Aspartylglucosaminuria in the United States

Hreidarsson, S., Thomas, G. H., Valle, D., Stevenson, R. E., Taylor, H., McCarty, J., Coker, S. B. & Green, W. R., 1983, In : Clinical Genetics. 23, 6, p. 427-435 9 p.

Research output: Contribution to journalArticle

Aspartylglucosaminuria
Intellectual Disability
Population
Differential Diagnosis

Cataract in gyrate atrophy: Clinical and morphologic studies

Kaiser-Kupfer, M., Kuwabara, T., Uga, S., Takki, K. & Valle, D., 1983, In : Investigative Ophthalmology and Visual Science. 24, 4, p. 432-436 5 p.

Research output: Contribution to journalArticle

Gyrate Atrophy
Cataract
Sutures
Lenses
Retinal Degeneration
1984

Defective imino acid metabolism in hypoglycin-treated rats

Goodman, S. I. & Valle, D., Feb 1984, In : Biochemical Medicine. 31, 1, p. 97-103 7 p.

Research output: Contribution to journalArticle

Proline Oxidase
Imino Acids
Metabolism
Oral Administration
Rats

Early manifestations of multiple sulfatase deficiency

Burk, R. D., Valle, D., Thomas, G. H., Miller, C., Moser, A. B., Moser, H. & Rosenbaum, K. N., 1984, In : Journal of Pediatrics. 104, 4, p. 574-578 5 p.

Research output: Contribution to journalArticle

Multiple Sulfatase Deficiency Disease
Fibroblasts
Metachromatic Leukodystrophy
Mucopolysaccharidoses
Sulfoglycosphingolipids

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

Lodato, R. F., Smith, R. J., Valle, D. & Crane, K., 1984, In : Journal of Cellular Physiology. 119, 1, p. 137-143 7 p.

Research output: Contribution to journalArticle

Azetidinecarboxylic Acid
Proline
Ornithine-Oxo-Acid Transaminase
Glutamic Acid
Cells

Phenotypic variability in siblings with Farber disease

Antonarakis, S. E., Valle, D., Moser, H. W., Moser, A. B., Qualman, S. J. & Zinkham, W. H., 1984, In : Journal of Pediatrics. 104, 3, p. 406-409 4 p.

Research output: Contribution to journalArticle

Farber Lipogranulomatosis
Sphingolipidoses
Amidohydrolases
Femur Head
Humerus