Page last updated: 2024-08-25

beta-hydroxyisovaleric acid and glycine

beta-hydroxyisovaleric acid has been researched along with glycine in 20 studies

Research

Studies (20)

Timeframe	Studies, this research(%)	All Research%
pre-1990	11 (55.00)	18.7374
1990's	3 (15.00)	18.2507
2000's	5 (25.00)	29.6817
2010's	1 (5.00)	24.3611
2020's	0 (0.00)	2.80

Authors

Authors	Studies
Holme, E; Jacobson, CE; Kristiansson, B	1
Bachmann, C; Dionisi-Vici, C; Graziani, MC; Sabetta, G	1
Mock, DM; Mock, NI; Weintraub, S	1
Bartlett, K; Cannon, RA; Conde, C; Gomez Vazquez, J; Lipson, M; Middleton, B; Nyhan, WL; Romanos, A; Sweetman, L	1
Inoue, F; Maltby, DA; Millington, DS; Roe, CR	1
Baker, H; Baswell, DL; Holman, RT; Mock, DM; Sweetman, L	1
Audenaert, SM; Squillaro, EJ; Wilson, WG	1
Hine, DG; Tanaka, K	1
Bartlett, K; Leonard, JV; Ng, H	1
Gregersen, N; Hobolth, N; Kølvraa, S; Lauritzen, R; Rasmussen, K; Rosleff, F	1
Inoue, Y; Kameyama, J; Momoi, T; Shigematsu, Y; Sudo, M; Suzuki, Y	1
Berry, SA; Nyhan, WL; Thuy, LP; Tuchman, M	1
Berry, SA; Gage, DA; Huang, ZH; Iwamoto, T; Johnston, K; Millington, DS; Packman, S; Röschinger, W; Sweetman, L; Yano, S	1
Bakkeren, J; Baumgartner, R; de Jong, J; Mourmans, J; Suormala, T; van Diggelen, OP; Wendel, U; Wevers, R	1
Almashanu, S; Baumgartner, ER; Baumgartner, MR; Dantas, MF; Fowler, B; Friebel, D; Gebhardt, B; Giunta, C; Hoffmann, GF; Suormala, T; Valle, D	1
Baumgartner, MR; Baykal, T; Can, G; Dantas, MF; Demir, F; Demirkol, M; Fowler, B; Gokcay, GH; Ince, Z	1
Barea, L; Baumgartner, MR; Dantas, MF; Fowler, B; Giugliani, R; Graziadio, C; Paskulin, G; Perla, A; Pinto, L; Rosa, R; Vargas, C; Wajner, M; Zen, P	1
Baumgartner, MR; Biberoglu, G; Demir, E; Eminoglu, FT; Hasanoglu, A; Okur, I; Ozcelik, AA; Tumer, L	1
Amaral, AU; Beskow, AP; da Silva, Lde B; Fernandes, CG; Leipnitz, G; Ribeiro, CA; Seminotti, B; Vargas, CR; Wajner, M; Zanatta, A	1
Afroze, B; Ali, ZZ; Fatimah, M; Jafri, L; Jamil, A; Khan, AH; Majid, H; Yusufzai, N	1

Other Studies

20 other study(ies) available for beta-hydroxyisovaleric acid and glycine

Article	Year
Biotin-responsive multiple carboxylase deficiency in an 8-year-old boy with normal serum biotinidase and fibroblast holocarboxylase-synthetase activities. Journal of inherited metabolic disease, 1988, Volume: 11, Issue:3 Topics: Amidohydrolases; Biotin; Biotinidase; Carbon-Nitrogen Ligases; Child; Fibroblasts; Glycine; Humans; Kinetics; Ligases; Male; Multiple Carboxylase Deficiency; Valerates	1988
Laryngeal stridor as a leading symptom in a biotinidase-deficient patient. Journal of inherited metabolic disease, 1988, Volume: 11, Issue:3 Topics: Amidohydrolases; Biotinidase; Female; Glycine; Humans; Infant; Lactates; Larynx; Propionates; Valerates	1988
Abnormal organic aciduria in biotin deficiency: the rat is similar to the human. The Journal of laboratory and clinical medicine, 1988, Volume: 112, Issue:2 Topics: Animals; Biotin; Disease Models, Animal; Gas Chromatography-Mass Spectrometry; Glycine; Male; Random Allocation; Rats; Rats, Inbred Strains; Valerates	1988
3-Ketothiolase deficiency. European journal of pediatrics, 1986, Volume: 144, Issue:6 Topics: 3-Hydroxybutyric Acid; Acetyl Coenzyme A; Acetyl-CoA C-Acyltransferase; Acyl Coenzyme A; Acyltransferases; Child; Female; Fibroblasts; Glycine; Humans; Hydroxybutyrates; Infant; Isoleucine; Ketosis; Male; Valerates	1986
Endogenous catabolism is the major source of toxic metabolites in isovaleric acidemia. The Journal of pediatrics, 1987, Volume: 110, Issue:1 Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Dietary Proteins; Glycine; Humans; Isovaleryl-CoA Dehydrogenase; Leucine; Male; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Valerates	1987
Biotin deficiency complicating parenteral alimentation: diagnosis, metabolic repercussions, and treatment. The Journal of pediatrics, 1985, Volume: 106, Issue:5 Topics: Alopecia; Biotin; Citrates; Diagnosis, Differential; Erythema; Fatty Acids, Essential; Female; Glycine; Humans; Infant; Isomerism; Lactates; Lactic Acid; Male; Nervous System Diseases; Parenteral Nutrition; Valerates; Zinc	1985
Hyperammonaemia in a preterm infant with isovaleric acidaemia. Journal of inherited metabolic disease, 1984, Volume: 7, Issue:2 Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Female; Glycine; Hemiterpenes; Humans; Infant, Newborn; Infant, Premature; Pentanoic Acids; Valerates	1984
The identification and the excretion pattern of isovaleryl glucuronide in the urine of patients with isovaleric acidemia. Pediatric research, 1984, Volume: 18, Issue:6 Topics: Acyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Chromatography, Gas; Gas Chromatography-Mass Spectrometry; Glucuronates; Glycine; Humans; Isovaleryl-CoA Dehydrogenase; Leucine; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Valerates	1984
A combined defect of three mitochondrial carboxylases presenting as biotin-responsive 3-methylcrotonyl glycinuria and 3-hydroxyisovaleric aciduria. Clinica chimica acta; international journal of clinical chemistry, 1980, Jan-15, Volume: 100, Issue:2 Topics: Acidosis; Acyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Biotin; Butyrates; Carbon Dioxide; Carboxy-Lyases; Child, Preschool; Crotonates; Female; Fibroblasts; Glycine; Humans; Hydroxy Acids; Ligases; Mitochondria; Propionates; Pyruvate Carboxylase Deficiency Disease; Valerates	1980
Non-ketotic C6-C10-dicarboxylic aciduria: biochemical investigations of two cases. Clinica chimica acta; international journal of clinical chemistry, 1980, Mar-28, Volume: 102, Issue:2-3 Topics: Adipates; Caproates; Caprylates; Child, Preschool; Chromatography, Gas; Decanoic Acids; Dicarboxylic Acids; Fatty Acid Desaturases; Fatty Acids; Glycine; Humans; Hydroxy Acids; Hydroxybutyrates; Hypoglycemia; Infant; Lipid Metabolism, Inborn Errors; Male; Mass Spectrometry; Valerates	1980
Changing plasma and urinary organic acid levels in a patient with isovaleric acidemia during an attack. Pediatric research, 1982, Volume: 16, Issue:9 Topics: Amino Acid Metabolism, Inborn Errors; Child, Preschool; Female; Glycine; Hemiterpenes; Humans; Hydroxybutyrates; Leucine; Pentanoic Acids; Valerates	1982
Partial methylcrotonyl-coenzyme A carboxylase deficiency in an infant with failure to thrive, gastrointestinal dysfunction, and hypertonia. Pediatrics, 1993, Volume: 91, Issue:3 Topics: Biotin; Carbon-Carbon Ligases; Failure to Thrive; Gastrointestinal Diseases; Glycine; Humans; Infant; Ligases; Male; Metabolism, Inborn Errors; Muscle Hypertonia; Valerates	1993
3-Hydroxyisovalerylcarnitine in patients with deficiency of 3-methylcrotonyl CoA carboxylase. Clinica chimica acta; international journal of clinical chemistry, 1995, Aug-31, Volume: 240, Issue:1 Topics: Carbon-Carbon Ligases; Carnitine; Child, Preschool; Female; Gas Chromatography-Mass Spectrometry; Glycine; Humans; Ligases; Male; Valerates	1995
Isolated (biotin-resistant) 3-methylcrotonyl-CoA carboxylase deficiency: four sibs devoid of pathology. Journal of inherited metabolic disease, 1995, Volume: 18, Issue:5 Topics: Carbon-Carbon Ligases; Carnitine; Child; Child, Preschool; Dietary Proteins; Female; Gas Chromatography-Mass Spectrometry; Glycine; Humans; Infant; Ligases; Male; Valerates	1995
Isolated 3-methylcrotonyl-CoA carboxylase deficiency: evidence for an allele-specific dominant negative effect and responsiveness to biotin therapy. American journal of human genetics, 2004, Volume: 75, Issue:5 Topics: Alleles; Base Sequence; Biotin; Carbon-Carbon Ligases; DNA Mutational Analysis; Dose-Response Relationship, Drug; Fibroblasts; Gene Expression; Genetic Vectors; Germany; Glycine; Greece; Humans; Infant, Newborn; Male; Mitochondrial Diseases; Molecular Sequence Data; Mutation; Reverse Transcriptase Polymerase Chain Reaction; Sequence Analysis, DNA; Transfection; Valerates	2004
Consanguineous 3-methylcrotonyl-CoA carboxylase deficiency: early-onset necrotizing encephalopathy with lethal outcome. Journal of inherited metabolic disease, 2005, Volume: 28, Issue:2 Topics: Carbon-Carbon Ligases; Consanguinity; Diagnosis, Differential; Fatal Outcome; Glycine; Humans; Infant; Infant, Newborn; Leukoencephalitis, Acute Hemorrhagic; Male; Metabolism, Inborn Errors; Mutation; RNA Splice Sites; Valerates	2005
Isolated 3-methylcrotonyl-coenzyme A carboxylase deficiency in a child with metabolic stroke. Journal of inherited metabolic disease, 2006, Volume: 29, Issue:1 Topics: Amino Acid Metabolism, Inborn Errors; Carbon-Carbon Ligases; Child, Preschool; Glycine; Humans; Male; Phenotype; Stroke; Valerates	2006
3-Methylcrotonyl-CoA carboxylase deficiency: phenotypic variability in a family. Journal of child neurology, 2009, Volume: 24, Issue:4 Topics: Adult; Brain; Carbon-Carbon Ligases; Carnitine; Cells, Cultured; Child, Preschool; DNA Mutational Analysis; Epilepsy; Fibroblasts; Gene Deletion; Genetic Predisposition to Disease; Genetic Testing; Genotype; Glycine; Humans; Infant; Language Development Disorders; Male; Mutation; Phenotype; Valerates	2009
Striatum is more vulnerable to oxidative damage induced by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency as compared to liver. International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience, 2009, Volume: 27, Issue:4 Topics: Animals; Corpus Striatum; Glutarates; Glutathione; Glycine; Humans; Liver; Male; Meglutol; Oxidative Stress; Oxo-Acid-Lyases; Rats; Rats, Wistar; Thiobarbituric Acid Reactive Substances; Valerates	2009
Diagnostic dilemma of patients with methylmalonic aciduria: Experience from a tertiary care centre in Pakistan. JPMA. The Journal of the Pakistan Medical Association, 2018, Volume: 68, Issue:4 Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Child, Preschool; Citrates; Cross-Sectional Studies; Female; Glycine; Humans; Infant; Lactic Acid; Male; Methionine; Mitochondrial Diseases; Pakistan; Tertiary Care Centers; Urinalysis; Valerates; Vitamin B 12 Deficiency	2018