valerates has been researched along with beta-methylcrotonylglycine in 14 studies
| Studies (valerates) | Trials (valerates) | Recent Studies (post-2010) (valerates) | Studies (beta-methylcrotonylglycine) | Trials (beta-methylcrotonylglycine) | Recent Studies (post-2010) (beta-methylcrotonylglycine) |
|---|---|---|---|---|---|
| 2,251 | 294 | 457 | 27 | 0 | 4 |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 5 (35.71) | 18.7374 |
| 1990's | 3 (21.43) | 18.2507 |
| 2000's | 5 (35.71) | 29.6817 |
| 2010's | 1 (7.14) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| 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 |
| Baker, H; Baswell, DL; Holman, RT; Mock, DM; Sweetman, L | 1 |
| Bartlett, K; Leonard, JV; Ng, H | 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 |
| Choi, JH; Jung, CW; Kim, GH; Kim, JH; Lee, BH; Lee, J; Yoo, HW | 1 |
14 other study(ies) available for valerates and beta-methylcrotonylglycine
| Article | Year |
|---|---|
Biotin-responsive multiple carboxylase deficiency in an 8-year-old boy with normal serum biotinidase and fibroblast holocarboxylase-synthetase activities.
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.
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.
Topics: Animals; Biotin; Disease Models, Animal; Gas Chromatography-Mass Spectrometry; Glycine; Male; Random Allocation; Rats; Rats, Inbred Strains; Valerates | 1988 |
Biotin deficiency complicating parenteral alimentation: diagnosis, metabolic repercussions, and treatment.
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 |
A combined defect of three mitochondrial carboxylases presenting as biotin-responsive 3-methylcrotonyl glycinuria and 3-hydroxyisovaleric aciduria.
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 |
Partial methylcrotonyl-coenzyme A carboxylase deficiency in an infant with failure to thrive, gastrointestinal dysfunction, and hypertonia.
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.
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.
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.
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.
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.
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.
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.
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 |
Uneventful clinical courses of Korean patients with methylcrotonylglycinuria and their common mutations.
Topics: Asian People; Carbon-Carbon Ligases; Child; Child, Preschool; Disease Progression; Female; Glycine; Humans; Infant; Infant, Newborn; Male; Mutation; Republic of Korea; Urea Cycle Disorders, Inborn; Valerates | 2012 |