Page last updated: 2024-09-05

beta-methylcrotonylglycine and glycine

beta-methylcrotonylglycine has been researched along with glycine in 27 studies

Compound Research Comparison

Studies
(beta-methylcrotonylglycine)
Trials
(beta-methylcrotonylglycine)
Recent Studies (post-2010)
(beta-methylcrotonylglycine)
Studies
(glycine)
Trials
(glycine)
Recent Studies (post-2010) (glycine)
270435,1637568,415

Protein Interaction Comparison

ProteinTaxonomybeta-methylcrotonylglycine (IC50)glycine (IC50)
Glutamate receptor ionotropic, NMDA 1 Rattus norvegicus (Norway rat)1.4852
Sodium- and chloride-dependent glycine transporter 1Homo sapiens (human)106
Glutamate receptor ionotropic, NMDA 2A Rattus norvegicus (Norway rat)0.3253
Glutamate receptor ionotropic, NMDA 2BRattus norvegicus (Norway rat)0.3253
Glutamate receptor ionotropic, NMDA 2CRattus norvegicus (Norway rat)0.3253
Glutamate receptor ionotropic, NMDA 2DRattus norvegicus (Norway rat)0.3253
Glutamate receptor ionotropic, NMDA 3BRattus norvegicus (Norway rat)0.3253
Glutamate receptor ionotropic, NMDA 3ARattus norvegicus (Norway rat)0.3253
Sodium- and chloride-dependent glycine transporter 2Homo sapiens (human)128

Research

Studies (27)

TimeframeStudies, this research(%)All Research%
pre-19909 (33.33)18.7374
1990's5 (18.52)18.2507
2000's9 (33.33)29.6817
2010's4 (14.81)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Hähnel, R; Wysocki, SJ1
Holme, E; Jacobson, CE; Kristiansson, B1
Bachmann, C; Dionisi-Vici, C; Graziani, MC; Sabetta, G1
Mock, DM; Mock, NI; Weintraub, S1
Baker, H; Baswell, DL; Holman, RT; Mock, DM; Sweetman, L1
Greter, J; Holme, E; Koivikko, M; Lindstedt, S1
Bartlett, K; Leonard, JV; Ng, H1
Borud, O; Chalmers, RA; Purkiss, P1
Baker, H; Nyhan, WL; Peterson, RM; Surh, L; Sweetman, L1
Berry, SA; Nyhan, WL; Thuy, LP; Tuchman, M1
Berry, SA; Gage, DA; Huang, ZH; Iwamoto, T; Johnston, K; Millington, DS; Packman, S; Röschinger, W; Sweetman, L; Yano, S1
Aleck, KA; Heidenreich, RA; Pearson, MA1
Bakkeren, J; Baumgartner, R; de Jong, J; Mourmans, J; Suormala, T; van Diggelen, OP; Wendel, U; Wevers, R1
Yoshida, I1
Ogier de Baulny, H; Saudubray, JM1
Mock, DM; Mock, NI1
Almashanu, S; Baumgartner, ER; Baumgartner, MR; Dantas, MF; Fowler, B; Friebel, D; Gebhardt, B; Giunta, C; Hoffmann, GF; Suormala, T; Valle, D1
Baumgartner, MR; Baykal, T; Can, G; Dantas, MF; Demir, F; Demirkol, M; Fowler, B; Gokcay, GH; Ince, Z1
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, P1
Barshop, BA; Baumgartner, MR; Chambaz, C; Cropcho, LJ; Finegold, DN; Gibson, KM; Koch, HG; Matern, D; Suormala, T; Vockley, J; Walters, N; Wolfe, LA1
Kluijtmans, LAJ; Koene, S; Mock, D; Morava, E; Pasch, M; Wevers, R1
Baumgartner, MR; Biberoglu, G; Demir, E; Eminoglu, FT; Hasanoglu, A; Okur, I; Ozcelik, AA; Tumer, L1
Amaral, AU; Beskow, AP; da Silva, Lde B; Fernandes, CG; Leipnitz, G; Ribeiro, CA; Seminotti, B; Vargas, CR; Wajner, M; Zanatta, A1
Busanello, EN; Moura, AP; Ribeiro, CA; Tonin, AM; Wajner, M; Zanatta, Â1
Choi, JH; Jung, CW; Kim, GH; Kim, JH; Lee, BH; Lee, J; Yoo, HW1
Baumgartner, MR; Burda, P; Bürer, C; Fowler, B; Grünert, SC; Morscher, RJ; Suormala, T1
Caruso, U; Cassanello, M; D'Onofrio, V; Enea, A; Garrone, G; Guala, G; Poma, F; Porta, F; Puccinelli, P; Santarelli, F; Spada, M1

Reviews

2 review(s) available for beta-methylcrotonylglycine and glycine

ArticleYear
[3-Methylcrotonyl-CoA carboxylase deficiency].
    Ryoikibetsu shokogun shirizu, 1998, Issue:18 Pt 1

    Topics: Amino Acid Metabolism, Inborn Errors; Carbon-Carbon Ligases; Diagnosis, Differential; Glycine; Humans; Leucine; Prognosis

1998
Branched-chain organic acidurias.
    Seminars in neonatology : SN, 2002, Volume: 7, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids, Branched-Chain; Diagnosis, Differential; Glycine; Hemiterpenes; Humans; Infant, Newborn; Malonates; Maple Syrup Urine Disease; Methylmalonic Acid; Pentanoic Acids; Prognosis; Propionates

2002

Other Studies

25 other study(ies) available for beta-methylcrotonylglycine and glycine

ArticleYear
3-Methylcrotonylglycine excretion in 3-hydroxy-3-methylglutaric aciduria.
    Clinica chimica acta; international journal of clinical chemistry, 1978, May-16, Volume: 86, Issue:1

    Topics: Crotonates; Glutarates; Glycine; Humans; Hydroxy Acids; Infant; Male; Metabolism, Inborn Errors

1978
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
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
Biotin-responsive 3-methylcrotonylglycinuria with biotinidase deficiency.
    Journal of inherited metabolic disease, 1985, Volume: 8 Suppl 2

    Topics: Amidohydrolases; Biotin; Biotinidase; Carbon-Carbon Ligases; Child, Preschool; Glycine; Humans; Ligases; Male

1985
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
Combined iminoglycinuria and cystine- and dibasic aminoaciduria in patients with propionic acidaemia and 3-methylcrotonylglycinuria.
    Journal of inherited metabolic disease, 1980, Volume: 3, Issue:3

    Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Amino Acids, Diamino; Child; Child, Preschool; Crotonates; Cystinuria; Glycine; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Propionates

1980
Clinical and metabolic abnormalities in a boy with dietary deficiency of biotin.
    Pediatrics, 1981, Volume: 68, Issue:4

    Topics: Alopecia; Biotin; Carbon-Carbon Ligases; Carboxy-Lyases; Child; Citrates; Diet; Egg Proteins; Facial Dermatoses; Glycine; Humans; Hydroxy Acids; Ligases; Male; Methylmalonyl-CoA Decarboxylase; Propionates; Skin Diseases

1981
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
Benign clinical presentation of 3-methylcrotonylglycinuria.
    Journal of inherited metabolic disease, 1995, Volume: 18, Issue:5

    Topics: Carbon-Carbon Ligases; Child, Preschool; Dietary Proteins; Glycine; Humans; Ligases; Male

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
Lymphocyte propionyl-CoA carboxylase is an early and sensitive indicator of biotin deficiency in rats, but urinary excretion of 3-hydroxypropionic acid is not.
    The Journal of nutrition, 2002, Volume: 132, Issue:7

    Topics: Animals; Biomarkers; Biotin; Carboxy-Lyases; Deficiency Diseases; Glycine; Lactic Acid; Lymphocytes; Male; Methylmalonyl-CoA Decarboxylase; Rats; Rats, Sprague-Dawley; Sensitivity and Specificity

2002
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
Potential misdiagnosis of 3-methylcrotonyl-coenzyme A carboxylase deficiency associated with absent or trace urinary 3-methylcrotonylglycine.
    Pediatrics, 2007, Volume: 120, Issue:5

    Topics: Carbon-Carbon Ligases; Child; Diagnostic Errors; Female; Glycine; Humans; Infant; Metabolism, Inborn Errors; Mutation, Missense

2007
Biotin-sensitive 3-methylcrotonylglycinuria in a patient with severe growth delay, ectodermal abnormalities, neonatal progeroid appearance, and developmental delay.
    Clinical dysmorphology, 2008, Volume: 17, Issue:3

    Topics: Abnormalities, Multiple; Aging, Premature; Biotin; Child, Preschool; Developmental Disabilities; Ectoderm; Female; Glycine; Growth Disorders; Humans; Metabolism, Inborn Errors

2008
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
3-Methylcrotonylglycine disrupts mitochondrial energy homeostasis and inhibits synaptic Na(+),K (+)-ATPase activity in brain of young rats.
    Cellular and molecular neurobiology, 2012, Volume: 32, Issue:2

    Topics: Animals; Antioxidants; Brain; Carbon Dioxide; Cerebral Cortex; Creatine Kinase; Cytosol; Electron Transport Chain Complex Proteins; Energy Metabolism; Glycine; Homeostasis; Mitochondria; Rats; Rats, Wistar; Sodium-Potassium-Exchanging ATPase; Synapses; Thiobarbituric Acid Reactive Substances

2012
Uneventful clinical courses of Korean patients with methylcrotonylglycinuria and their common mutations.
    Journal of human genetics, 2012, Volume: 57, Issue:1

    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
A single mutation in MCCC1 or MCCC2 as a potential cause of positive screening for 3-methylcrotonyl-CoA carboxylase deficiency.
    Molecular genetics and metabolism, 2012, Volume: 105, Issue:4

    Topics: Acyl Coenzyme A; Carbon-Carbon Ligases; Carnitine; Cells, Cultured; Child; Child, Preschool; DNA Mutational Analysis; Female; Fibroblasts; Glycine; Heterozygote; Humans; Infant; Infant, Newborn; Male; Mutation; Neonatal Screening; Real-Time Polymerase Chain Reaction; Skin; Urea Cycle Disorders, Inborn

2012
A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency.
    Italian journal of pediatrics, 2013, May-24, Volume: 39

    Topics: Acetyl-CoA C-Acetyltransferase; Acidosis; Acute Disease; Amino Acid Metabolism, Inborn Errors; Glutarates; Glycine; Humans; Hypoglycemia; Infant, Newborn; Male; Meglutol

2013