6.4.1.4: methylcrotonoyl-CoA carboxylase

This is an abbreviated version!
For detailed information about methylcrotonoyl-CoA carboxylase, go to the full flat file.

Word Map on EC 6.4.1.4

Reaction

ATP
+
3-methylcrotonoyl-CoA
+
HCO3-
+
H+
=
ADP
+
phosphate
+
3-methylglutaconyl-CoA

Synonyms

Carboxylase, methylcrotonyl coenzyme A, Methylcrotonyl coenzyme A carboxylase, beta-Methylcrotonyl coenzyme A carboxylase, beta-methylcrotonyl CoA carboxylase, Mcase, 3-methylcrotonyl-coenzyme A carboxylase, MCCase, 3-methylcrotonyl-CoA carboxylase, glutaconyl-CoA decarboxylase, glutaconyl-CoA:biotin carboxytransferase, methylcrotonyl-CoA carboxylase, beta-methylcrotonyl-CoA carboxylase, beta-MCC, 3-methylcrotonyl-CoA:carbon-dioxide ligase (ADP-forming), MCC, MCCC, 3-methylcrotonyl coenzyme A carboxylase, MC-CoA carboxylase, 3-methylcrotonyl CoA carboxylase, 3-MCC, MCCA, MCCB, PaMCC, 3-methylcrotonyl-coenzyme A, Pa-MCCase, 3-methylcrotonyl coenzyme-A carboxylase, liuD, liuB, Mcc2

ECTree

     6 Ligases
         6.4 Forming carbon-carbon bonds
             6.4.1 Ligases that form carbon-carbon bonds (only sub-subclass identified to date)
                6.4.1.4 methylcrotonoyl-CoA carboxylase

Reference

Reference on EC 6.4.1.4 - methylcrotonoyl-CoA carboxylase

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REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Chandler, C.S.; Ballard, F.J.
Distribution and degradation of biotin-containing carboxylases in human cell lines
Biochem. J.
232
385-393
1985
Homo sapiens
Manually annotated by BRENDA team
Lau, E.P.; Cochran, B.C.; Munson, L.; Fall, R.R.
Bovine kidney 3-methylcrotonyl-CoA and propionyl-CoA carboxylase: each enzyme contains nonidentical subunits
Proc. Natl. Acad. Sci. USA
76
214-218
1979
Bos taurus
Manually annotated by BRENDA team
Schiele, U.; Lynen, F.
3-Methylcrotonyl-CoA carboxylase from Achromobacter
Methods Enzymol.
71
781-791
1981
Achromobacter sp.
-
Manually annotated by BRENDA team
Hector, M.L.; Cochran, B.C.; Logue, E.A.; Fall, R.R.
Subcellular localization of methylcrotonyl-coenzyme A carboxylase in bovine kidney
Arch. Biochem. Biophys.
199
28-36
1980
Bos taurus
Manually annotated by BRENDA team
Lau, E.P.; Cochran, B.C.; Fall, R.R.
Isolation of 3-methylcrotonyl-coenzyme A carboxylase from bovine kidney
Arch. Biochem. Biophys.
205
352-359
1980
Bos taurus
Manually annotated by BRENDA team
Lau, E.P.; Fall, R.R.
3-Methylcrotonyl-CoA carboxylase from bovine kidney. EC 6.4.1.4. 3-Methylcrotonyl-CoA: carbon dioxide ligase (ADP-forming)
Methods Enzymol.
71
800-804
1981
Bos taurus, Homo sapiens, Rattus norvegicus
Manually annotated by BRENDA team
Fall, R.R.
3-Methylcrotonyl-CoA and geranyl-CoA carboxylases from Pseudomonas citronellolis
Methods Enzymol.
71
791-799
1981
Pseudomonas citronellolis
Manually annotated by BRENDA team
Fall, R.R.; Hector, M.L.
Acyl-coenzyme A carboxylases. Homologous 3-methylcrotonyl-CoA and geranyl-CoA carboxylase from Pseudomonas citronellolis
Biochemistry
16
4000-4005
1977
Pseudomonas citronellolis
Manually annotated by BRENDA team
Hector, M.L.; Fall, R.R.
Evidence for distinct 3-methylcrotonyl-CoA and geranyl-CoA carboxylases in Pseudomonas citronellolis
Biochem. Biophys. Res. Commun.
71
746-753
1976
Pseudomonas citronellolis
Manually annotated by BRENDA team
Schiele, U.; Niedermeier, R.; Sturzer, M.; Lynen, F.
Investigations of the structure of 3-methylcrotonyl-CoA carboxylase from Achromobacter
Eur. J. Biochem.
60
259-266
1975
Achromobacter sp., Achromobacter sp. IVS
Manually annotated by BRENDA team
Ghneim, H.K.; Noy, G.; Bartlett, K.
Biotin-dependent carboxylases and cultured human fibroblasts
Biochem. Soc. Trans.
9
405-406
1981
Homo sapiens
-
Manually annotated by BRENDA team
Clauss, M.; Motel, A.; Lichtenthaler, H.K.
Studies on the plant methylcrotonyl-CoA carboxylase
J. Plant Physiol.
141
508-511
1993
Hordeum vulgare, Lens culinaris, Pisum sativum, Zea mays
-
Manually annotated by BRENDA team
Alban, C.; Baldet, P.; Axiotis, S.; Douce, R.
Purification and characterization of 3-methylcrotonyl-coenzyme A carboxylase from higher plant mitochondria
Plant Physiol.
102
957-965
1993
Pisum sativum, Solanum tuberosum
Manually annotated by BRENDA team
Baldet, P.; Alban, C.; Axiotis, S.; Douce, R.
Characterization of biotin and 3-methylcrotonyl-coenzyme A carboxylase in higher plant mitochondria
Plant Physiol.
99
450-455
1992
Pisum sativum
Manually annotated by BRENDA team
Diez, T.A.; Wurtele, E.S.; Nikolau, B.J.
Purification and characterization of 3-methylcrotonyl-coenzyme-A carboxylase from leaves of Zea mays
Arch. Biochem. Biophys.
310
64-75
1994
Zea mays
Manually annotated by BRENDA team
Lau, E.P.; Gibson, K.M.; Fall, R.R.
Alternate microbial strategies for the metabolism of a 3-methyl branched alkanoic acid
Curr. Microbiol.
4
163-167
1980
Arthrobacter sp., Mycobacterium sp., Pseudomonas citronellolis
-
Manually annotated by BRENDA team
Weaver, L.M.; Lebrun, L.; Franklin, A.; Huang, L.; Hoffman, N.; Wurtele, E.S.; Nikolau, B.J.
Molecular cloning of the biotinylated subunit of 3-methylcrotonyl-coenzyme A carboxylase of Arabidopsis thaliana
Plant Physiol.
107
1013-1014
1995
Arabidopsis thaliana
Manually annotated by BRENDA team
Wang, X.; Wurtele, E.S.; Keller, G.; McKean, A.L.; Nikolau, B.J.
Molecular cloning of cDNAs and genes coding for beta-methylcrotonyl-CoA carboxylase of tomato
J. Biol. Chem.
269
11760-11768
1994
Solanum lycopersicum
Manually annotated by BRENDA team
Braune, A.; Bendrat, K.; Rospert, S.; Buckel, W.
The sodium ion translocating glutaconyl-CoA decarboxylase from Acidaminococcus fermentans: cloning and function of the genes forming a second operon
Mol. Microbiol.
31
473-487
1999
Acidaminococcus fermentans
Manually annotated by BRENDA team
McKean, A.L.; Ke, J.; Song, J.; Che, P.; Achenbach, S.; Nikolau, B.J.; Wurtele, E.S.
Molecular characterization of the non-biotin-containing subunit of 3-methylcrotonyl-CoA carboxylase
J. Biol. Chem.
275
5582-5590
2000
Arabidopsis thaliana (Q9LDD8)
Manually annotated by BRENDA team
Bixel, M.G.; Hamprecht, B.
Immunocytochemical localization of beta-methylcrotonyl-CoA carboxylase in astroglial cells and neurons in culture
J. Neurochem.
74
1059-1067
2000
Rattus norvegicus, Rattus norvegicus Wistar
Manually annotated by BRENDA team
Wiedmann, S.; Eudy, J.D.; Zempleni, J.
Biotin supplementation increases expression of genes encoding interferon-gamma, interleukin-1beta, and 3-methylcrotonyl-CoA carboxylase, and decreases expression of the gene encoding interleukin-4 in human peripheral blood mononuclear cells
J. Nutr.
133
716-719
2003
Homo sapiens
Manually annotated by BRENDA team
Maier, A.; Lichtenthaler, H.K.
Methylcrotonyl-CoA carboxylase from barley (Hordeum vulgare). Purification, kinetic characterization, and senescence-related induction of activity
J. Plant Physiol.
152
213-221
1998
Hordeum vulgare
-
Manually annotated by BRENDA team
Wurtele, E.S.; Nikolau, B.J.
Characterization of 3-methylcrotonyl-CoA carboxylase from plants
Methods Enzymol.
324
280-292
2000
Daucus carota, Glycine max, Hordeum vulgare, Solanum lycopersicum, Pisum sativum, Solanum tuberosum, Zea mays
Manually annotated by BRENDA team
Anderson, M.D.; Che, P.; Song, J.; Nikolau, B.J.; Wurtele, E.S.
3-Methylcrotonyl-coenzyme A carboxylase is a component of the mitochondrial leucine catabolic pathway in plants
Plant Physiol.
118
1127-1138
1998
Glycine max
Manually annotated by BRENDA team
Che, P.; Wurtele, E.S.; Nikolau, B.J.
Metabolic and environmental regulation of 3-methylcrotonyl-coenzyme A carboxylase expression in Arabidopsis
Plant Physiol.
129
625-637
2002
Arabidopsis thaliana (Q42523)
Manually annotated by BRENDA team
Che, P.; Weaver, L.M.; Wurtele, E.S.; Nikolau, B.J.
The role of biotin in regulating 3-methylcrotonyl-coenzyme a carboxylase expression in Arabidopsis
Plant Physiol.
131
1479-1486
2003
Arabidopsis sp.
Manually annotated by BRENDA team
Sloane, V.; Waldrop, G.L.
Kinetic characterization of mutations found in propionic acidemia and methylcrotonylglycinuria: Evidence for cooperativity in biotin carboxylase
J. Biol. Chem.
279
15772-15778
2004
Escherichia coli
Manually annotated by BRENDA team
Stadler, S.C.; Polanetz, R.; Meier, S.; Mayerhofer, P.U.; Herrmann, J.M.; Anslinger, K.; Roscher, A.A.; Roeschinger, W.; Holzinger, A.
Mitochondrial targeting signals and mature peptides of 3-methylcrotonyl-CoA carboxylase
Biochem. Biophys. Res. Commun.
334
939-946
2005
Homo sapiens
Manually annotated by BRENDA team
Rodriguez, J.M.; Ruiz-Sala, P.; Ugarte, M.; Penalva, M.A.
Fungal metabolic model for 3-methylcrotonyl-CoA carboxylase deficiency
J. Biol. Chem.
279
4578-4587
2004
Aspergillus nidulans
Manually annotated by BRENDA team
Baykal, T.; Gokcay, G.H.; Ince, Z.; Dantas, M.F.; Fowler, B.; Baumgartner, M.R.; Demir, F.; Can, G.; Demirkol, M.
Consanguineous 3-methylcrotonyl-CoA carboxylase deficiency: early-onset necrotizing encephalopathy with lethal outcome
J. Inherit. Metab. Dis.
28
229-233
2005
Homo sapiens
Manually annotated by BRENDA team
Baumgartner, M.R.
Molecular mechanism of dominant expression in 3-methylcrotonyl-CoA carboxylase deficiency
J. Inherit. Metab. Dis.
28
301-309
2005
Homo sapiens
Manually annotated by BRENDA team
Abe, S.; Chamnan, C.; Miyamoto, K.; Minamino, Y.; Nouda, M.
Isolation and identification of 3-methylcrotonyl coenzyme A carboxylase cDNAs and pyruvate carboxylase, and their expression in red seabream (Pagrus major) organs
Mar. Biotechnol.
6
527-540
2004
Pagrus major
-
Manually annotated by BRENDA team
Hoschle, B.; Gnau, V.; Jendrossek, D.
Methylcrotonyl-CoA and geranyl-CoA carboxylases are involved in leucine/isovalerate utilization (Liu) and acyclic terpene utilization (Atu), and are encoded by liuB/liuD and atuC/atuF, in Pseudomonas aeruginosa
Microbiology
151
3649-3656
2005
Pseudomonas aeruginosa, Pseudomonas aeruginosa PAo1 Smr ins-atuF
Manually annotated by BRENDA team
Murin, R.; Verleysdonk, S.; Rapp, M.; Hamprecht, B.
Immunocytochemical localization of 3-methylcrotonyl-CoA carboxylase in cultured ependymal, microglial and oligodendroglial cells
J. Neurochem.
97
1393-1402
2006
Rattus norvegicus (Q5I0C3)
Manually annotated by BRENDA team
Chu, C.H.; Cheng, D.
Expression, purification, characterization of human 3-methylcrotonyl-CoA carboxylase (MCCC)
Protein Expr. Purif.
53
421-427
2007
Homo sapiens
Manually annotated by BRENDA team
Aguilar, J.A.; Diaz-Perez, C.; Diaz-Perez, A.L.; Rodriguez-Zavala, J.S.; Nikolau, B.J.; Campos-Garcia, J.
Substrate specificity of the 3-methylcrotonyl coenzyme A (CoA) and geranyl-CoA carboxylases from Pseudomonas aeruginosa
J. Bacteriol.
190
4888-4893
2008
Pseudomonas aeruginosa
Manually annotated by BRENDA team
Mock, D.M.
Marginal biotin deficiency is common in normal human pregnancy and is highly teratogenic in mice
J. Nutr.
139
154-157
2009
Homo sapiens, Mus musculus
Manually annotated by BRENDA team
Arnold, G.L.; Koeberl, D.D.; Matern, D.; Barshop, B.; Braverman, N.; Burton, B.; Cederbaum, S.; Fiegenbaum, A.; Garganta, C.; Gibson, J.; Goodman, S.I.; Harding, C.; Kahler, S.; Kronn, D.; Longo, N.
A Delphi-based consensus clinical practice protocol for the diagnosis and management of 3-methylcrotonyl CoA carboxylase deficiency
Mol. Genet. Metab.
93
363-370
2008
Homo sapiens
Manually annotated by BRENDA team
Stucki, M.; Suormala, T.; Fowler, B.; Valle, D.; Baumgartner, M.R.
Cryptic exon activation by disruption of exon splice enhancer: novel mechanism causing 3-methylcrotonyl-CoA carboxylase deficiency
J. Biol. Chem.
284
28953-28957
2009
Homo sapiens
Manually annotated by BRENDA team
Eminoglu, F.T.; Ozcelik, A.A.; Okur, I.; Tumer, L.; Biberoglu, G.; Demir, E.; Hasanoglu, A.; Baumgartner, M.R.
3-Methylcrotonyl-CoA carboxylase deficiency: phenotypic variability in a family
J. Child Neurol.
24
478-481
2009
Homo sapiens
Manually annotated by BRENDA team
Horvath, T.; Stratton, S.; Bogusiewicz, A.; Owen, S.; Mock, D.; Moran, J.
Quantitative measurement of urinary excretion of 3-hydroxyisovaleryl carnitine by LC-MS/MS as an indicator of biotin status in humans
Anal. Chem.
82
9543-9548
2010
Homo sapiens
Manually annotated by BRENDA team
Nguyen, K.; Naviaux, R.; Patra, S.; Barshop, B.; Nyhan, W.
Novel mutations in the human MCCA and MCCB gene causing methylcrotonylglycinuria
Mol. Genet. Metab.
102
218-221
2011
Homo sapiens
Manually annotated by BRENDA team
Diaz-Perez, C.; Diaz-Perez, A.L.; Rodriguez-Zavala, J.S.; Campos-Garcia, J.
Structural evidence for the involvement of the residues Ser187 and Tyr422 in substrate recognition in the 3-methylcrotonyl-coenzyme A carboxylase from Pseudomonas aeruginosa
J. Biochem.
154
291-297
2013
Pseudomonas aeruginosa (Q9I299 and Q9I297), Pseudomonas aeruginosa
Manually annotated by BRENDA team
Huang, C.S.; Ge, P.; Zhou, Z.H.; Tong, L.
An unanticipated architecture of the 750-kDa holoenzyme of 3-methylcrotonyl-CoA carboxylase
Nature
481
219-223
2012
Pseudomonas aeruginosa (Q9I299 and Q9I297), Pseudomonas aeruginosa
Manually annotated by BRENDA team
Ding, G.; Che, P.; Ilarslan, H.; Wurtele, E.S.; Nikolau, B.J.
Genetic dissection of methylcrotonyl CoA carboxylase indicates a complex role for mitochondrial leucine catabolism during seed development and germination
Plant J.
70
562-577
2012
Arabidopsis thaliana (Q42523), Arabidopsis thaliana (Q9LDD8)
Manually annotated by BRENDA team
Diaz-Perez, C.; Rodriguez-Zavala, J.S.; Diaz-Perez, A.L.; Campos-Garcia, J.
Co-expression of alpha and beta subunits of the 3-methylcrotonyl-coenzyme A carboxylase from Pseudomonas aeruginosa
World J. Microbiol. Biotechnol.
28
1185-1191
2012
Pseudomonas aeruginosa (Q9I299 and Q9I297), Pseudomonas aeruginosa
Manually annotated by BRENDA team
Jurado, A.; Huang, C.; Zhang, X.; Zhou, Z.; Tong, L.
Structure and substrate selectivity of the 750-kDa alpha6beta6 holoenzyme of geranyl-CoA carboxylase
Nat. Commun.
6
8986
2015
Pseudomonas aeruginosa (Q9I297), Pseudomonas aeruginosa DSM 22644 (Q9I297)
Manually annotated by BRENDA team
Ge, F.; Huang, W.; Chen, Z.; Zhang, C.; Xiong, Q.; Bowler, C.; Yang, J.; Xu, J.; Hu, H.
Methylcrotonyl-CoA carboxylase regulates triacylglycerol accumulation in the model diatom Phaeodactylum tricornutum
Plant Cell
26
1681-1697
2014
Phaeodactylum tricornutum
Manually annotated by BRENDA team