proline and BCKD Deficiency

proline has been researched along with BCKD Deficiency in 14 studies

Research

Studies (14)

TimeframeStudies, this research(%)All Research%
pre-199014 (100.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
EFRON, ML; MACCREADY, RA; MOSER, HW; YOUNG, D1
Moser, HW; Prensky, AL1
Bremer, HJ; de Bree, PK; Duran, M; Kamerling, JP; Przyrembel, H; Vliegenthart, JF; Wadman, SK1
Meister, A; Wellner, D1
Milner, RD; Wirdnam, PK1
Reavey, PC; Yadav, GC1
Ampola, MG1
Frimpter, GW1
Kroll, S; Toussaint, W; Zebisch, P2
Ampola, MG; Efron, ML1
Menne, F1
Buravina, TA; Iur'eva, EA; Koroleva, IA; Lebedev, VP; Mukhina, IuG1
Knoll, E; Scheibenreiter, S; Schmierer, G; Schön, R; Thalhammer, O1

Reviews

6 review(s) available for proline and BCKD Deficiency

ArticleYear
A survey of inborn errors of amino acid metabolism and transport in man.
    Annual review of biochemistry, 1981, Volume: 50

    Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Amino Acids, Branched-Chain; Biological Transport; Cystinosis; Female; Glutathione; Glycine; Homocystinuria; Humans; Hydroxyproline; Infant; Infant, Newborn; Lysine; Maple Syrup Urine Disease; Phenylalanine; Proline; Serine; Tyrosine; Urea; Valine

1981
Phenylketonuria and other disorders of amino acid metabolism.
    Pediatric clinics of North America, 1973, Volume: 20, Issue:2

    Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Child; Child, Preschool; Counseling; Cystathionine; Cystinosis; Cystinuria; Diet Therapy; Dietary Proteins; Family; Female; Hartnup Disease; Histidine; Homocystinuria; Humans; Hyperglycemia; Infant; Infant, Newborn; Lysine; Maple Syrup Urine Disease; Ornithine; Phenylketonurias; Pregnancy; Proline; Tyrosine

1973
Aminoacidurias due to inherited disorders of metabolism. 2.
    The New England journal of medicine, 1973, Oct-25, Volume: 289, Issue:17

    Topics: Acidosis; Alanine; Amino Acid Metabolism, Inborn Errors; Arginase; Arginine; Carbamates; Citrulline; Dipeptides; Glutarates; Glycine; Histidine; Humans; Hydroxyproline; Hyperglycemia; Infant, Newborn; Keto Acids; Lysine; Malonates; Maple Syrup Urine Disease; Ornithine Carbamoyltransferase; Phosphotransferases; Proline; Sarcosine; Succinates; Urea; Valine

1973
The aminoacidurias.
    Pediatric clinics of North America, 1967, Volume: 14, Issue:4

    Topics: Amino Acid Metabolism, Inborn Errors; Glycine; Hartnup Disease; Histidine; Homocystinuria; Humans; Hydroxyproline; Lysine; Maple Syrup Urine Disease; Methionine; Phenylketonurias; Proline; Tyrosine

1967
[Feeble mindedness caused by genetic disorders of amino acid metabolism].
    Hippokrates, 1968, May-31, Volume: 39, Issue:10

    Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Citrulline; Glycine; Hartnup Disease; Histidine; Homocystinuria; Humans; Hydroxyproline; Intellectual Disability; Lysine; Maple Syrup Urine Disease; Methionine; Phenylketonurias; Proline; Sarcosine; Succinates; Transferases; Tryptophan; Valine

1968
[Primary and secondary hyperaminoaciduria in children (review of the literature)].
    Voprosy okhrany materinstva i detstva, 1970, Volume: 15, Issue:11

    Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Biological Transport, Active; Cystinuria; Fanconi Syndrome; Glycine; Glycosuria, Renal; Hartnup Disease; Histidine; Humans; Infant, Newborn; Kidney Diseases; Kidney Tubules; Maple Syrup Urine Disease; Phenylketonurias; Proline; Renal Aminoacidurias; Renal Tubular Transport, Inborn Errors; Sodium

1970

Other Studies

8 other study(ies) available for proline and BCKD Deficiency

ArticleYear
A SIMPLE CHROMATOGRAPHIC SCREENING TEST FOR THE DETECTION OF DISORDERS OF AMINO ACID METABOLISM. A TECHNIC USING WHOLE BLOOD OR URINE COLLECTED ON FILTER PAPER.
    The New England journal of medicine, 1964, Jun-25, Volume: 270

    Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Blood Chemical Analysis; Chromatography; Citrulline; Glycine; Histidine; Homocysteine; Humans; Hydroxyproline; Intellectual Disability; Maple Syrup Urine Disease; Metabolic Diseases; Phenylketonurias; Proline; Proteins; Renal Aminoacidurias; Urine

1964
Changes in the amino acid composition of proteolipids of white matter during maturation of the human nervous system.
    Journal of neurochemistry, 1967, Volume: 14, Issue:1

    Topics: Adult; Aged; Amino Acids; Aspartic Acid; Central Nervous System; Child; Frontal Lobe; Humans; Infant; Infant, Newborn; Leucine; Lipoproteins; Maple Syrup Urine Disease; Phenylalanine; Proline; Tyrosine

1967
Azetidine-2-carboxylic acid contaminated dietary proline as a cause of urinary excretion of 4-amino-2-(S-cysteinyl)butyric acid in patients on oral treatment with a synthetic diet.
    Journal of chromatography, 1983, Oct-14, Volume: 277

    Topics: Azetidinecarboxylic Acid; Azetines; Biotransformation; Chromatography, Thin Layer; Dipeptides; Female; Food Contamination; Food, Formulated; Humans; Infant; Male; Maple Syrup Urine Disease; Proline

1983
The pancreatic beta cell fraction in children with errors of amino acid metabolism.
    Pediatric research, 1982, Volume: 16, Issue:3

    Topics: Amino Acid Metabolism, Inborn Errors; Child; Cystinosis; Homocystinuria; Humans; Infant; Infant, Newborn; Islets of Langerhans; Maple Syrup Urine Disease; Methionine; Phenylketonurias; Proline; Tyrosine

1982
Aminoacidopathies: a review of 3 years experience of investigations in a Kuwait hospital.
    Journal of inherited metabolic disease, 1988, Volume: 11, Issue:3

    Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Amino Acids; Child; Child, Preschool; Citrulline; Cystinuria; Female; Glycine; Homocystinuria; Humans; Infant; Infant, Newborn; Kuwait; Male; Maple Syrup Urine Disease; Phenylalanine; Phenylketonurias; Proline; Tyrosine

1988
[Hereditary amino acid metabolism disorders. Indications for early diagnosis].
    Fortschritte der Medizin, 1972, Apr-13, Volume: 90, Issue:11

    Topics: Albinism; Alkaptonuria; Amino Acid Metabolism, Inborn Errors; Amino Acids; Arginine; Cystathionine; Cystinosis; Diagnosis, Differential; Glycine; Hartnup Disease; Histidine; Homocystinuria; Humans; Infant; Infant, Newborn; Maple Syrup Urine Disease; Methods; Methylmalonic Acid; Phenylketonurias; Proline; Propionates; Tyrosine

1972
[Diagnosis of inborn amino acid metabolism errors. Important symptoms and laboratory methods].
    Fortschritte der Medizin, 1972, Apr-13, Volume: 90, Issue:11

    Topics: Albinism; Alkaptonuria; Amino Acid Metabolism, Inborn Errors; Amino Acids; Arginine; Clinical Laboratory Techniques; Cystathionine; Cystinosis; Glycine; Hartnup Disease; Histidine; Homocystinuria; Humans; Maple Syrup Urine Disease; Methods; Methylmalonic Acid; Phenylketonurias; Proline; Propionates; Tyrosine

1972
[5 years of the Austrian program for the early detection of inborn errors of metabolism. Activity report].
    Wiener klinische Wochenschrift, 1972, Volume: 84

    Topics: Amino Acid Metabolism, Inborn Errors; Austria; Child, Preschool; Chromatography, Thin Layer; Diet Therapy; Economics, Medical; Follow-Up Studies; Galactosemias; Glycine; Histidine; Homocystinuria; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Maple Syrup Urine Disease; Metabolism, Inborn Errors; Metal Metabolism, Inborn Errors; Multiphasic Screening; Phenylketonurias; Proline; Time Factors; Tyrosine

1972