glycine has been researched along with BCKD Deficiency in 26 studies
Excerpt | Relevance | Reference |
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" Maple syrup urine disease (MSUD), isovaleric acidaemia (IVA), propionic aciduria (PA) and methylmalonic aciduria (MMA) represent the most commonly encountered abnormal organic acidurias." | 4.81 | Branched-chain organic acidurias. ( Ogier de Baulny, H; Saudubray, JM, 2002) |
"N-Lactylvaline, N-lactylleucine, N-lactylisoleucine and the N-2-hydroxyisovaleryl conjugates of glycine, valine, leucine and isoleucine have been identified in urine from a patient with the intermediate type of maple syrup urine disease." | 3.67 | New conjugated urinary metabolites in intermediate type maple syrup urine disease. ( Hagenfeldt, L; Naglo, AS, 1987) |
" The patients suffered from distinct variants of maple syrup urine disease, propionic acidaemia, methylmalonic acidaemia, lactic acidosis and hyperglycinuria." | 3.66 | [Human fibroblast bank for studying amino acid disorders and organic acidemias]. ( del Valle, JA; Merinero, B; Pérez-Cerdá, C; Ugarte, M, 1982) |
"Leucine metabolism in cultured skin fibroblasts from patients with isovaleric acidemia was compared with that in normal fibroblasts and in cells from patients with maple syrup urine disease using [1-(14)C] and [2-(14)C] leucine as substrates." | 3.65 | Metabolism of [1-(14)C] and [2-(14)C] leucine in cultured skin fibroblasts from patients with isovaleric acidemia. Characterization of metabolic defects. ( Mandell, R; Shih, VE; Tanaka, K, 1976) |
"Neonatal seizures, as distinguished from nonconvulsive abnormal movements, are a significant problem in neonatal intensive care units." | 2.37 | Neonatal seizures. ( Bergman, I; Crumrine, P; Painter, MJ, 1986) |
Timeframe | Studies, this research(%) | All Research% |
---|---|---|
pre-1990 | 24 (92.31) | 18.7374 |
1990's | 0 (0.00) | 18.2507 |
2000's | 2 (7.69) | 29.6817 |
2010's | 0 (0.00) | 24.3611 |
2020's | 0 (0.00) | 2.80 |
Authors | Studies |
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EFRON, ML | 3 |
YOUNG, D | 1 |
MOSER, HW | 1 |
MACCREADY, RA | 1 |
Brautigam, CA | 1 |
Chuang, JL | 1 |
Tomchick, DR | 1 |
Machius, M | 1 |
Chuang, DT | 1 |
Wellner, D | 1 |
Meister, A | 1 |
del Valle, JA | 1 |
Merinero, B | 1 |
Pérez-Cerdá, C | 1 |
Ugarte, M | 1 |
Ogier de Baulny, H | 1 |
Saudubray, JM | 1 |
Brady, RO | 1 |
Omenn, GS | 1 |
Tanaka, K | 1 |
Mandell, R | 1 |
Shih, VE | 1 |
Ota, K | 1 |
Yadav, GC | 1 |
Reavey, PC | 1 |
Painter, MJ | 1 |
Bergman, I | 1 |
Crumrine, P | 1 |
Hagenfeldt, L | 1 |
Naglo, AS | 1 |
Levy, HL | 2 |
Frimpter, GW | 1 |
Martin, JJ | 1 |
Schlote, W | 1 |
Kroll, S | 2 |
Zebisch, P | 2 |
Toussaint, W | 2 |
Tomaszewski, L | 1 |
Ampola, MG | 1 |
Menne, F | 1 |
Lebedev, VP | 1 |
Iur'eva, EA | 1 |
Mukhina, IuG | 1 |
Buravina, TA | 1 |
Koroleva, IA | 1 |
Thalhammer, O | 1 |
Scheibenreiter, S | 1 |
Schön, R | 1 |
Knoll, E | 1 |
Schmierer, G | 1 |
Barkin, E | 1 |
Sietti, C | 1 |
Raven, EJ | 1 |
11 reviews available for glycine and BCKD Deficiency
Article | Year |
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AMINOACIDURIA.
Topics: Amino Acid Metabolism, Inborn Errors; Diagnosis; Glycine; Humans; Kidney; Leucine; Maple Syrup Urine | 1965 |
A survey of inborn errors of amino acid metabolism and transport in man.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Amino Acids, Branched-Chain; Biological Transport | 1981 |
Branched-chain organic acidurias.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids, Branched-Chain; Diagnosis, Differential; Glycine; | 2002 |
Inborn errors of metabolism: clues to understanding human behavioral disorders.
Topics: Adrenal Hyperplasia, Congenital; Brain; Galactosemias; Glycine; Hepatolenticular Degeneration; Heter | 1976 |
[Vitamin dependency].
Topics: Anemia, Sideroblastic; Animals; Biotin; Crotonates; Cystathionine; gamma-Aminobutyric Acid; Glutamat | 1975 |
Neonatal seizures.
Topics: Adrenoleukodystrophy; Amino Acid Metabolism, Inborn Errors; Ammonia; Anesthetics, Local; Anticonvuls | 1986 |
Genetic screening.
Topics: Amino Acid Metabolism, Inborn Errors; Anemia, Sickle Cell; Arginine; Carbohydrate Metabolism, Inborn | 1973 |
Aminoacidurias due to inherited disorders of metabolism. 2.
Topics: Acidosis; Alanine; Amino Acid Metabolism, Inborn Errors; Arginase; Arginine; Carbamates; Citrulline; | 1973 |
The aminoacidurias.
Topics: Amino Acid Metabolism, Inborn Errors; Glycine; Hartnup Disease; Histidine; Homocystinuria; Humans; H | 1967 |
[Feeble mindedness caused by genetic disorders of amino acid metabolism].
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Citrulline; Glycine; Hartnup Disease; Histi | 1968 |
[Primary and secondary hyperaminoaciduria in children (review of the literature)].
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Biological Transport, Active; Cystinuria; Fanconi | 1970 |
15 other studies available for glycine and BCKD Deficiency
Article | Year |
---|---|
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.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Blood Chemical Analysis; Chromatography; Citrulli | 1964 |
Crystal structure of human dihydrolipoamide dehydrogenase: NAD+/NADH binding and the structural basis of disease-causing mutations.
Topics: Binding Sites; Catalysis; Cloning, Molecular; Crystallography, X-Ray; Databases, Protein; Dihydrolip | 2005 |
[Human fibroblast bank for studying amino acid disorders and organic acidemias].
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Cell Line; Cells, Cultured; Child; Child, Preschool; | 1982 |
Inherited metabolic diseases of the nervous system.
Topics: Amino Acid Metabolism, Inborn Errors; Aspartylglucosaminuria; Carbohydrate Metabolism, Inborn Errors | 1976 |
Metabolism of [1-(14)C] and [2-(14)C] leucine in cultured skin fibroblasts from patients with isovaleric acidemia. Characterization of metabolic defects.
Topics: 2-Methyl-4-chlorophenoxyacetic Acid; Amino Acid Metabolism, Inborn Errors; Carbon Dioxide; Cell Line | 1976 |
Aminoacidopathies: a review of 3 years experience of investigations in a Kuwait hospital.
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Amino Acids; Child; Child, Preschool; Citrulline; | 1988 |
New conjugated urinary metabolites in intermediate type maple syrup urine disease.
Topics: Amino Acids; Amino Acids, Branched-Chain; Carboxylic Acids; Female; Gas Chromatography-Mass Spectrom | 1987 |
Neuropathological study of aminoacidurias.
Topics: Age Factors; Amino Acid Metabolism, Inborn Errors; Central Nervous System; Cystathionine; Female; Gl | 1972 |
[Hereditary amino acid metabolism disorders. Indications for early diagnosis].
Topics: Albinism; Alkaptonuria; Amino Acid Metabolism, Inborn Errors; Amino Acids; Arginine; Cystathionine; | 1972 |
[Diagnosis of inborn amino acid metabolism errors. Important symptoms and laboratory methods].
Topics: Albinism; Alkaptonuria; Amino Acid Metabolism, Inborn Errors; Amino Acids; Arginine; Clinical Labora | 1972 |
[The inborn errors of metabolism of amino acids].
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Chemical Phenomena; Chemistry; Cystinuria; Glycin | 1973 |
[5 years of the Austrian program for the early detection of inborn errors of metabolism. Activity report].
Topics: Amino Acid Metabolism, Inborn Errors; Austria; Child, Preschool; Chromatography, Thin Layer; Diet Th | 1972 |
Comparison of amino acid concentrations between plasma and erythrocytes. Studies in normal human subjects and those with metabolic disorders.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids; Arginine; Aspartic Acid; Biological Transp | 1971 |
[Paper chromatography in the detection of aminoacidopathies].
Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Chromatography, Paper; Glycine; Histidin | 1971 |
[Progressive encephalopathy after a symptom-free period in the newborn infant, due to disorders of amino acid metabolism. Illustrated with case histories of patients with hyperglycinemia and maple syrup urine disease].
Topics: Amino Acid Metabolism, Inborn Errors; Female; Glycine; Humans; Infant; Infant, Newborn; Infant, Newb | 1969 |