GENTAUR Belgium BVBA BE0473327336 Voortstraat 49, 1910 Kampenhout BELGIUM Tel 0032 16 58 90 45
GENTAUR U.S.A Genprice Inc,Logistics 547 Yurok Circle, SanJose, CA 95123
Tel (408) 780-0908, Fax (408) 780-0908, [email protected]

Did you know ? If you order before Friday 14h we deliver 90PCT of the the time next Tuesday, Gentaur another in time delivery

Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCase subunit beta) (EC 6.4.1.4) (3-methylcrotonyl-CoA carboxylase 2) (3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit) (3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta)

 MCCB_HUMAN              Reviewed;         563 AA.
Q9HCC0; A6NIY9; Q96C27; Q9Y4L7;
05-MAR-2002, integrated into UniProtKB/Swiss-Prot.
01-MAR-2001, sequence version 1.
03-JUL-2019, entry version 179.
RecName: Full=Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial;
Short=MCCase subunit beta;
EC=6.4.1.4 {ECO:0000269|PubMed:17360195};
AltName: Full=3-methylcrotonyl-CoA carboxylase 2;
AltName: Full=3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit;
AltName: Full=3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta;
Flags: Precursor;
Name=MCCC2; Synonyms=MCCB;
Homo sapiens (Human).
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini;
Catarrhini; Hominidae; Homo.
NCBI_TaxID=9606;
[1]
NUCLEOTIDE SEQUENCE [GENOMIC DNA / MRNA] (ISOFORM 1).
Fukuda T., Otsuka H., Morishita R., Takemoto Y., Sone M., Nakao M.,
Abe S., Kondo I.;
"Human non-biotin containing subunit gene of 3-methylcrotonyl-CoA
carboxylase (MCCB).";
Submitted (OCT-2000) to the EMBL/GenBank/DDBJ databases.
[2]
NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), SUBCELLULAR LOCATION, AND
VARIANTS MCC2D ARG-167 AND THR-218.
PubMed=11170888; DOI=10.1086/318202;
Gallardo M.E., Desviat L.R., Rodriguez J.M., Esparza-Gordillo J.,
Perez-Cerda C., Perez B., Rodriguez-Pombo P., Criado O., Sanz R.,
Morton D.H., Gibson K.M., Le T.P., Ribes A., Rodriguez de Cordoba S.,
Ugarte M., Penalva M.A.;
"The molecular basis of 3-methylcrotonylglycinuria, a disorder of
leucine catabolism.";
Am. J. Hum. Genet. 68:334-346(2001).
[3]
NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), AND VARIANTS MCC2D GLN-99;
GLN-155; LEU-173; CYS-193; ARG-310 AND MET-339.
PubMed=11181649; DOI=10.1172/JCI11948;
Baumgartner M.R., Almashanu S., Suormala T., Obie C., Cole R.N.,
Packman S., Baumgartner E.R., Valle D.;
"The molecular basis of human 3-methylcrotonyl-CoA carboxylase
deficiency.";
J. Clin. Invest. 107:495-504(2001).
[4]
NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), AND VARIANT MCC2D THR-268.
PubMed=11406611; DOI=10.1093/hmg/10.12.1299;
Holzinger A., Roeschinger W., Lagler F., Mayerhofer P.U., Lichtner P.,
Kattenfeld T., Thuy L.P., Nyhan W.L., Koch H.G., Muntau A.C.,
Roscher A.A.;
"Cloning of the human MCCA and MCCB genes and mutations therein reveal
the molecular cause of 3-methylcrotonyl-CoA: carboxylase deficiency.";
Hum. Mol. Genet. 10:1299-1306(2001).
[5]
NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
PubMed=15372022; DOI=10.1038/nature02919;
Schmutz J., Martin J., Terry A., Couronne O., Grimwood J., Lowry S.,
Gordon L.A., Scott D., Xie G., Huang W., Hellsten U., Tran-Gyamfi M.,
She X., Prabhakar S., Aerts A., Altherr M., Bajorek E., Black S.,
Branscomb E., Caoile C., Challacombe J.F., Chan Y.M., Denys M.,
Detter J.C., Escobar J., Flowers D., Fotopulos D., Glavina T.,
Gomez M., Gonzales E., Goodstein D., Grigoriev I., Groza M.,
Hammon N., Hawkins T., Haydu L., Israni S., Jett J., Kadner K.,
Kimball H., Kobayashi A., Lopez F., Lou Y., Martinez D., Medina C.,
Morgan J., Nandkeshwar R., Noonan J.P., Pitluck S., Pollard M.,
Predki P., Priest J., Ramirez L., Retterer J., Rodriguez A.,
Rogers S., Salamov A., Salazar A., Thayer N., Tice H., Tsai M.,
Ustaszewska A., Vo N., Wheeler J., Wu K., Yang J., Dickson M.,
Cheng J.-F., Eichler E.E., Olsen A., Pennacchio L.A., Rokhsar D.S.,
Richardson P., Lucas S.M., Myers R.M., Rubin E.M.;
"The DNA sequence and comparative analysis of human chromosome 5.";
Nature 431:268-274(2004).
[6]
NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L.,
Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R.,
Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V.,
Hannenhalli S., Turner R., Yooseph S., Lu F., Nusskern D.R.,
Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H.,
Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G.,
Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W.,
Venter J.C.;
Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases.
[7]
NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 2).
TISSUE=Testis, and Uterus;
PubMed=15489334; DOI=10.1101/gr.2596504;
The MGC Project Team;
"The status, quality, and expansion of the NIH full-length cDNA
project: the Mammalian Gene Collection (MGC).";
Genome Res. 14:2121-2127(2004).
[8]
PROTEIN SEQUENCE OF 23-28, AND SUBCELLULAR LOCATION.
TISSUE=Kidney;
PubMed=16023992; DOI=10.1016/j.bbrc.2005.06.190;
Stadler S.C., Polanetz R., Meier S., Mayerhofer P.U., Herrmann J.M.,
Anslinger K., Roscher A.A., Roschinger W., Holzinger A.;
"Mitochondrial targeting signals and mature peptides of 3-
methylcrotonyl-CoA carboxylase.";
Biochem. Biophys. Res. Commun. 334:939-946(2005).
[9]
FUNCTION, CATALYTIC ACTIVITY, BIOPHYSICOCHEMICAL PROPERTIES, AND
PATHWAY.
PubMed=17360195; DOI=10.1016/j.pep.2007.01.012;
Chu C.H., Cheng D.;
"Expression, purification, characterization of human 3-methylcrotonyl-
CoA carboxylase (MCCC).";
Protein Expr. Purif. 53:421-427(2007).
[10]
NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 469-563.
The European IMAGE consortium;
Submitted (JUN-1999) to the EMBL/GenBank/DDBJ databases.
[11]
IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
PubMed=21269460; DOI=10.1186/1752-0509-5-17;
Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P.,
Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.;
"Initial characterization of the human central proteome.";
BMC Syst. Biol. 5:17-17(2011).
[12]
IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
TISSUE=Liver;
PubMed=24275569; DOI=10.1016/j.jprot.2013.11.014;
Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D.,
Wang L., Ye M., Zou H.;
"An enzyme assisted RP-RPLC approach for in-depth analysis of human
liver phosphoproteome.";
J. Proteomics 96:253-262(2014).
[13]
IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
PubMed=25944712; DOI=10.1002/pmic.201400617;
Vaca Jacome A.S., Rabilloud T., Schaeffer-Reiss C., Rompais M.,
Ayoub D., Lane L., Bairoch A., Van Dorsselaer A., Carapito C.;
"N-terminome analysis of the human mitochondrial proteome.";
Proteomics 15:2519-2524(2015).
[14]
VARIANTS MCC2D ARG-190; VAL-218 AND TYR-280.
PubMed=17968484; DOI=10.1007/s10038-007-0211-9;
Uematsu M., Sakamoto O., Sugawara N., Kumagai N., Morimoto T.,
Yamaguchi S., Hasegawa Y., Kobayashi H., Ihara K., Yoshino M.,
Watanabe Y., Inokuchi T., Yokoyama T., Kiwaki K., Nakamura K.,
Endo F., Tsuchiya S., Ohura T.;
"Novel mutations in five Japanese patients with 3-methylcrotonyl-CoA
carboxylase deficiency.";
J. Hum. Genet. 52:1040-1043(2007).
[15]
VARIANTS MCC2D GLN-99; TRP-155; GLN-155; TYR-190; THR-268; ARG-282;
ARG-310; PHE-375 AND VAL-456, AND CHARACTERIZATION OF VARIANTS TYR-190
AND ARG-352.
PubMed=16010683; DOI=10.1002/humu.9352;
Dantas M.F., Suormala T., Randolph A., Coelho D., Fowler B., Valle D.,
Baumgartner M.R.;
"3-Methylcrotonyl-CoA carboxylase deficiency: mutation analysis in 28
probands, 9 symptomatic and 19 detected by newborn screening.";
Hum. Mutat. 26:164-174(2005).
[16]
VARIANTS MCC2D PHE-355; ARG-477; ARG-517 AND SER-520.
PubMed=21071250; DOI=10.1016/j.ymgme.2010.10.008;
Nguyen K.V., Naviaux R.K., Patra S., Barshop B.A., Nyhan W.L.;
"Novel mutations in the human MCCA and MCCB gene causing
methylcrotonylglycinuria.";
Mol. Genet. Metab. 102:218-221(2011).
[17]
VARIANTS MCC2D TYR-280 AND SER-459.
PubMed=22150417; DOI=10.1111/j.1399-0004.2011.01704.x;
Cho S.Y., Park H.D., Lee Y.W., Ki C.S., Lee S.Y., Sohn Y.B.,
Park S.W., Kim S.H., Ji S., Kim S.J., Choi E.W., Kim C.H., Ko A.R.,
Paik K.H., Lee D.H., Jin D.K.;
"Mutational spectrum in eight Korean patients with 3-methylcrotonyl-
CoA carboxylase deficiency.";
Clin. Genet. 81:96-98(2012).
[18]
VARIANTS MCC2D VAL-218; MET-339 AND GLY-523.
PubMed=22264772; DOI=10.1016/j.ymgme.2011.12.018;
Morscher R.J., Grunert S.C., Burer C., Burda P., Suormala T.,
Fowler B., Baumgartner M.R.;
"A single mutation in MCCC1 or MCCC2 as a potential cause of positive
screening for 3-methylcrotonyl-CoA carboxylase deficiency.";
Mol. Genet. Metab. 105:602-606(2012).
[19]
VARIANTS MCC2D PHE-39; GLN-99; PHE-101; GLY-118 DEL; PHE-131; ASN-146;
THR-152; TRP-155; ARG-167; ASP-169; LEU-173; ARG-190; TYR-190;
CYS-193; HIS-193; ASN-200; THR-218; VAL-218; GLU-220; LEU-224;
ASP-237; LEU-266; TYR-280; ARG-282; ARG-310; MET-339; VAL-340 ARG-352;
PHE-355; PHE-375; THR-403; LEU-434; VAL-456; ARG-475; ARG-477;
ARG-517; SER-520; GLY-523 AND GLU-555, AND CHARACTERIZATION OF
VARIANTS MCC2D PHE-39; GLY-118 DEL; ASN-146; ARG-282; LEU-434;
VAL-456; ARG-475 AND GLY-523.
PubMed=22642865; DOI=10.1186/1750-1172-7-31;
Gruenert S.C., Stucki M., Morscher R.J., Suormala T., Buerer C.,
Burda P., Christensen E., Ficicioglu C., Herwig J., Koelker S.,
Moeslinger D., Pasquini E., Santer R., Schwab K.O., Wilcken B.,
Fowler B., Yue W.W., Baumgartner M.R.;
"3-methylcrotonyl-CoA carboxylase deficiency: clinical, biochemical,
enzymatic and molecular studies in 88 individuals.";
Orphanet J. Rare Dis. 7:31-54(2012).
[20]
VARIANTS MCC2D ILE-139 AND ARG-319.
PubMed=25382614; DOI=10.1111/cge.12535;
Yang L., Yang J., Zhang T., Weng C., Hong F., Tong F., Yang R.,
Yin X., Yu P., Huang X., Qi M.;
"Identification of eight novel mutations and transcript analysis of
two splicing mutations in Chinese newborns with MCC deficiency.";
Clin. Genet. 88:484-488(2015).
[21]
VARIANTS MCC2D VAL-68; ARG-105; TRP-155; ASP-163; ASN-200; ALA-214;
TRP-216; THR-218; ASP-230; CYS-318; MET-339; VAL-387; PRO-393;
ARG-410; ASP-410; THR-441; VAL-461; ARG-475 AND THR-524.
PubMed=27601257; DOI=10.1016/j.gene.2016.09.003;
Fonseca H., Azevedo L., Serrano C., Sousa C., Marcao A., Vilarinho L.;
"3-Methylcrotonyl-CoA carboxylase deficiency: Mutational spectrum
derived from comprehensive newborn screening.";
Gene 594:203-210(2016).
-!- FUNCTION: Carboxyltransferase subunit of the 3-methylcrotonyl-CoA
carboxylase, an enzyme that catalyzes the conversion of 3-
methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for
leucine and isovaleric acid catabolism.
{ECO:0000269|PubMed:17360195}.
-!- CATALYTIC ACTIVITY:
Reaction=3-methylbut-2-enoyl-CoA + ATP + hydrogencarbonate = ADP +
H(+) + phosphate + trans-3-methylglutaconyl-CoA;
Xref=Rhea:RHEA:13589, ChEBI:CHEBI:15378, ChEBI:CHEBI:17544,
ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:57344,
ChEBI:CHEBI:57346, ChEBI:CHEBI:456216; EC=6.4.1.4;
Evidence={ECO:0000269|PubMed:17360195};
-!- BIOPHYSICOCHEMICAL PROPERTIES:
Kinetic parameters:
KM=45 uM for ATP {ECO:0000269|PubMed:17360195};
KM=74 uM for 3-methylcrotonyl-CoA {ECO:0000269|PubMed:17360195};
Note=kcat is 4.0 sec(-1).;
-!- PATHWAY: Amino-acid degradation; L-leucine degradation; (S)-3-
hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 2/3.
{ECO:0000269|PubMed:17360195}.
-!- SUBUNIT: Probably a dodecamer composed of six biotin-containing
alpha subunits (MCCC1) and six beta (MCCC2) subunits.
-!- SUBCELLULAR LOCATION: Mitochondrion matrix
{ECO:0000269|PubMed:11170888, ECO:0000269|PubMed:16023992}.
-!- ALTERNATIVE PRODUCTS:
Event=Alternative splicing; Named isoforms=2;
Name=1;
IsoId=Q9HCC0-1; Sequence=Displayed;
Name=2;
IsoId=Q9HCC0-2; Sequence=VSP_000069;
Note=No experimental confirmation available.;
-!- DISEASE: 3-methylcrotonoyl-CoA carboxylase 2 deficiency (MCC2D)
[MIM:210210]: An autosomal recessive disorder of leucine
catabolism. The phenotype is variable, ranging from neonatal onset
with severe neurological involvement to asymptomatic adults. There
is a characteristic organic aciduria with massive excretion of 3-
hydroxyisovaleric acid and 3-methylcrotonylglycine, usually in
combination with a severe secondary carnitine deficiency.
{ECO:0000269|PubMed:11170888, ECO:0000269|PubMed:11181649,
ECO:0000269|PubMed:11406611, ECO:0000269|PubMed:16010683,
ECO:0000269|PubMed:17968484, ECO:0000269|PubMed:21071250,
ECO:0000269|PubMed:22150417, ECO:0000269|PubMed:22264772,
ECO:0000269|PubMed:22642865, ECO:0000269|PubMed:25382614,
ECO:0000269|PubMed:27601257}. Note=The disease is caused by
mutations affecting the gene represented in this entry.
-!- SIMILARITY: Belongs to the AccD/PCCB family. {ECO:0000305}.
-!- SEQUENCE CAUTION:
Sequence=AAH14897.1; Type=Frameshift; Positions=359; Evidence={ECO:0000305};
-----------------------------------------------------------------------
Copyrighted by the UniProt Consortium, see https://www.uniprot.org/terms
Distributed under the Creative Commons Attribution (CC BY 4.0) License
-----------------------------------------------------------------------
EMBL; AB050049; BAB16880.1; -; mRNA.
EMBL; AB050050; BAB41121.1; -; Genomic_DNA.
EMBL; AF310971; AAG53094.1; -; mRNA.
EMBL; AF301000; AAK16404.1; -; mRNA.
EMBL; AF261884; AAK49409.1; -; mRNA.
EMBL; AC138832; -; NOT_ANNOTATED_CDS; Genomic_DNA.
EMBL; CH471084; EAW95693.1; -; Genomic_DNA.
EMBL; BC014897; AAH14897.1; ALT_FRAME; mRNA.
EMBL; BC065027; AAH65027.1; -; mRNA.
EMBL; AL079298; CAB45194.1; -; mRNA.
CCDS; CCDS34184.1; -. [Q9HCC0-1]
RefSeq; NP_071415.1; NM_022132.4. [Q9HCC0-1]
RefSeq; XP_005248624.1; XM_005248567.1.
SMR; Q9HCC0; -.
BioGrid; 122050; 46.
CORUM; Q9HCC0; -.
IntAct; Q9HCC0; 27.
MINT; Q9HCC0; -.
STRING; 9606.ENSP00000343657; -.
DrugBank; DB00121; Biotin.
iPTMnet; Q9HCC0; -.
PhosphoSitePlus; Q9HCC0; -.
BioMuta; MCCC2; -.
DMDM; 20138731; -.
REPRODUCTION-2DPAGE; IPI00784044; -.
EPD; Q9HCC0; -.
jPOST; Q9HCC0; -.
MaxQB; Q9HCC0; -.
PaxDb; Q9HCC0; -.
PeptideAtlas; Q9HCC0; -.
PRIDE; Q9HCC0; -.
ProteomicsDB; 81665; -.
ProteomicsDB; 81666; -. [Q9HCC0-2]
Ensembl; ENST00000340941; ENSP00000343657; ENSG00000131844. [Q9HCC0-1]
GeneID; 64087; -.
KEGG; hsa:64087; -.
UCSC; uc003kbs.5; human. [Q9HCC0-1]
CTD; 64087; -.
DisGeNET; 64087; -.
GeneCards; MCCC2; -.
HGNC; HGNC:6937; MCCC2.
HPA; HPA038300; -.
HPA; HPA038301; -.
HPA; HPA061546; -.
MalaCards; MCCC2; -.
MIM; 210210; phenotype.
MIM; 609014; gene.
neXtProt; NX_Q9HCC0; -.
OpenTargets; ENSG00000131844; -.
Orphanet; 6; 3-methylcrotonyl-CoA carboxylase deficiency.
PharmGKB; PA30681; -.
eggNOG; KOG0540; Eukaryota.
eggNOG; COG4799; LUCA.
GeneTree; ENSGT00940000155949; -.
HOGENOM; HOG000218692; -.
InParanoid; Q9HCC0; -.
KO; K01969; -.
OMA; AYLPIMS; -.
OrthoDB; 632665at2759; -.
PhylomeDB; Q9HCC0; -.
TreeFam; TF300446; -.
BioCyc; MetaCyc:ENSG00000131844-MONOMER; -.
Reactome; R-HSA-196780; Biotin transport and metabolism.
Reactome; R-HSA-3371599; Defective HLCS causes multiple carboxylase deficiency.
Reactome; R-HSA-70895; Branched-chain amino acid catabolism.
UniPathway; UPA00363; UER00861.
GenomeRNAi; 64087; -.
PRO; PR:Q9HCC0; -.
Proteomes; UP000005640; Chromosome 5.
Bgee; ENSG00000131844; Expressed in 207 organ(s), highest expression level in epithelium of mammary gland.
ExpressionAtlas; Q9HCC0; baseline and differential.
Genevisible; Q9HCC0; HS.
GO; GO:0002169; C:3-methylcrotonyl-CoA carboxylase complex, mitochondrial; TAS:ParkinsonsUK-UCL.
GO; GO:0005829; C:cytosol; TAS:Reactome.
GO; GO:1905202; C:methylcrotonoyl-CoA carboxylase complex; IDA:ParkinsonsUK-UCL.
GO; GO:0005759; C:mitochondrial matrix; IDA:ParkinsonsUK-UCL.
GO; GO:0005739; C:mitochondrion; IDA:ParkinsonsUK-UCL.
GO; GO:0005524; F:ATP binding; IEA:UniProtKB-KW.
GO; GO:0004485; F:methylcrotonoyl-CoA carboxylase activity; IEA:UniProtKB-EC.
GO; GO:0006768; P:biotin metabolic process; TAS:Reactome.
GO; GO:0009083; P:branched-chain amino acid catabolic process; TAS:Reactome.
GO; GO:0015936; P:coenzyme A metabolic process; IEA:Ensembl.
GO; GO:0006552; P:leucine catabolic process; IBA:GO_Central.
GO; GO:0051291; P:protein heterooligomerization; NAS:ParkinsonsUK-UCL.
InterPro; IPR034733; AcCoA_carboxyl.
InterPro; IPR029045; ClpP/crotonase-like_dom_sf.
InterPro; IPR011763; COA_CT_C.
InterPro; IPR011762; COA_CT_N.
Pfam; PF01039; Carboxyl_trans; 1.
SUPFAM; SSF52096; SSF52096; 2.
PROSITE; PS50989; COA_CT_CTER; 1.
PROSITE; PS50980; COA_CT_NTER; 1.
1: Evidence at protein level;
Acetylation; Alternative splicing; ATP-binding; Complete proteome;
Direct protein sequencing; Disease mutation; Ligase; Mitochondrion;
Nucleotide-binding; Reference proteome; Transit peptide.
TRANSIT 1 22 Mitochondrion.
{ECO:0000269|PubMed:16023992}.
CHAIN 23 563 Methylcrotonoyl-CoA carboxylase beta
chain, mitochondrial.
/FTId=PRO_0000000291.
DOMAIN 49 306 CoA carboxyltransferase N-terminal.
{ECO:0000255|PROSITE-ProRule:PRU01136}.
DOMAIN 309 555 CoA carboxyltransferase C-terminal.
{ECO:0000255|PROSITE-ProRule:PRU01137}.
REGION 49 555 Carboxyltransferase.
{ECO:0000255|PROSITE-ProRule:PRU01138}.
REGION 343 372 Acyl-CoA binding. {ECO:0000255}.
MOD_RES 70 70 N6-acetyllysine; alternate.
{ECO:0000250|UniProtKB:Q3ULD5}.
MOD_RES 70 70 N6-succinyllysine; alternate.
{ECO:0000250|UniProtKB:Q3ULD5}.
MOD_RES 141 141 N6-succinyllysine.
{ECO:0000250|UniProtKB:Q3ULD5}.
MOD_RES 495 495 N6-acetyllysine; alternate.
{ECO:0000250|UniProtKB:Q3ULD5}.
MOD_RES 495 495 N6-succinyllysine; alternate.
{ECO:0000250|UniProtKB:Q3ULD5}.
MOD_RES 511 511 N6-acetyllysine.
{ECO:0000250|UniProtKB:Q3ULD5}.
VAR_SEQ 209 246 Missing (in isoform 2).
{ECO:0000303|PubMed:15489334}.
/FTId=VSP_000069.
VARIANT 39 39 S -> F (in MCC2D; has some wild-type
residual activity; dbSNP:rs398124371).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072507.
VARIANT 68 68 G -> V (in MCC2D; unknown pathological
significance; dbSNP:rs1187203558).
{ECO:0000269|PubMed:27601257}.
/FTId=VAR_077291.
VARIANT 99 99 E -> Q (in MCC2D; severe and mild form;
dbSNP:rs119103219).
{ECO:0000269|PubMed:11181649,
ECO:0000269|PubMed:16010683,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_012792.
VARIANT 101 101 S -> F (in MCC2D; dbSNP:rs748028684).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072508.
VARIANT 105 105 G -> R (in MCC2D; unknown pathological
significance).
{ECO:0000269|PubMed:27601257}.
/FTId=VAR_077292.
VARIANT 118 118 Missing (in MCC2D; has some wild-type
residual activity).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072509.
VARIANT 131 131 C -> F (in MCC2D).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072510.
VARIANT 139 139 T -> I (in MCC2D).
{ECO:0000269|PubMed:25382614}.
/FTId=VAR_077293.
VARIANT 146 146 Y -> N (in MCC2D; has some wild-type
residual activity).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072511.
VARIANT 152 152 K -> T (in MCC2D).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072512.
VARIANT 155 155 R -> Q (in MCC2D; mild form;
dbSNP:rs119103220).
{ECO:0000269|PubMed:11181649,
ECO:0000269|PubMed:16010683}.
/FTId=VAR_012793.
VARIANT 155 155 R -> W (in MCC2D; dbSNP:rs141030969).
{ECO:0000269|PubMed:16010683,
ECO:0000269|PubMed:22642865,
ECO:0000269|PubMed:27601257}.
/FTId=VAR_072513.
VARIANT 163 163 N -> D (in MCC2D; unknown pathological
significance).
{ECO:0000269|PubMed:27601257}.
/FTId=VAR_077294.
VARIANT 167 167 C -> R (in MCC2D; dbSNP:rs119103222).
{ECO:0000269|PubMed:11170888,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_012794.
VARIANT 169 169 Y -> D (in MCC2D).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072514.
VARIANT 173 173 S -> L (in MCC2D; severe form;
dbSNP:rs752866557).
{ECO:0000269|PubMed:11181649,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_012795.
VARIANT 190 190 H -> R (in MCC2D; dbSNP:rs119103225).
{ECO:0000269|PubMed:17968484,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_072515.
VARIANT 190 190 H -> Y (in MCC2D; produces severely
decreased wild-type residual activity;
dbSNP:rs773774134).
{ECO:0000269|PubMed:16010683,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_072516.
VARIANT 193 193 R -> C (in MCC2D; mild form;
dbSNP:rs547662164).
{ECO:0000269|PubMed:11181649,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_012796.
VARIANT 193 193 R -> H (in MCC2D; dbSNP:rs535519604).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072517.
VARIANT 200 200 I -> N (in MCC2D; unknown pathological
significance; dbSNP:rs140806722).
{ECO:0000269|PubMed:22642865,
ECO:0000269|PubMed:27601257}.
/FTId=VAR_072518.
VARIANT 214 214 G -> A (in MCC2D; dbSNP:rs277995).
{ECO:0000269|PubMed:27601257}.
/FTId=VAR_077295.
VARIANT 216 216 C -> W (in MCC2D).
{ECO:0000269|PubMed:27601257}.
/FTId=VAR_077296.
VARIANT 218 218 A -> T (in MCC2D; dbSNP:rs886043524).
{ECO:0000269|PubMed:11170888,
ECO:0000269|PubMed:22642865,
ECO:0000269|PubMed:27601257}.
/FTId=VAR_012797.
VARIANT 218 218 A -> V (in MCC2D; dbSNP:rs760420191).
{ECO:0000269|PubMed:17968484,
ECO:0000269|PubMed:22264772,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_072519.
VARIANT 220 220 G -> E (in MCC2D; dbSNP:rs1254750166).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072520.
VARIANT 224 224 P -> L (in MCC2D; dbSNP:rs1195601465).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072521.
VARIANT 230 230 N -> D (in MCC2D; dbSNP:rs766753795).
{ECO:0000269|PubMed:27601257}.
/FTId=VAR_077297.
VARIANT 237 237 G -> D (in MCC2D).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072522.
VARIANT 266 266 H -> L (in MCC2D).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072523.
VARIANT 268 268 R -> T (in MCC2D; asymptomatic form;
dbSNP:rs119103223).
{ECO:0000269|PubMed:11406611,
ECO:0000269|PubMed:16010683}.
/FTId=VAR_012798.
VARIANT 268 268 Missing (in MCC2D).
{ECO:0000269|PubMed:16010683}.
/FTId=VAR_072524.
VARIANT 280 280 D -> Y (in MCC2D; dbSNP:rs119103226).
{ECO:0000269|PubMed:17968484,
ECO:0000269|PubMed:22150417,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_067199.
VARIANT 282 282 H -> R (in MCC2D; has some wild-type
residual activity).
{ECO:0000269|PubMed:16010683,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_072525.
VARIANT 310 310 P -> R (in MCC2D; mild form;
dbSNP:rs119103221).
{ECO:0000269|PubMed:11181649,
ECO:0000269|PubMed:16010683,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_012799.
VARIANT 318 318 Y -> C (in MCC2D; dbSNP:rs773115035).
{ECO:0000269|PubMed:27601257}.
/FTId=VAR_077298.
VARIANT 319 319 G -> R (in MCC2D; dbSNP:rs1443551700).
{ECO:0000269|PubMed:25382614}.
/FTId=VAR_077299.
VARIANT 339 339 V -> M (in MCC2D; severe form;
dbSNP:rs150591260).
{ECO:0000269|PubMed:11181649,
ECO:0000269|PubMed:22264772,
ECO:0000269|PubMed:22642865,
ECO:0000269|PubMed:27601257}.
/FTId=VAR_012800.
VARIANT 340 340 D -> V (in MCC2D; dbSNP:rs398124370).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072526.
VARIANT 352 352 G -> R (in MCC2D; produces severely
decreased wild-type residual activity;
dbSNP:rs765438239).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072527.
VARIANT 355 355 L -> F (in MCC2D; dbSNP:rs757052602).
{ECO:0000269|PubMed:21071250,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_072528.
VARIANT 375 375 V -> F (in MCC2D).
{ECO:0000269|PubMed:16010683,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_072529.
VARIANT 387 387 F -> V (in MCC2D; unknown pathological
significance; dbSNP:rs1450515408).
{ECO:0000269|PubMed:27601257}.
/FTId=VAR_077300.
VARIANT 393 393 Q -> P (in MCC2D; dbSNP:rs750782118).
{ECO:0000269|PubMed:27601257}.
/FTId=VAR_077301.
VARIANT 403 403 N -> T (in MCC2D; dbSNP:rs142887940).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072530.
VARIANT 410 410 G -> D (in MCC2D; dbSNP:rs771440617).
{ECO:0000269|PubMed:27601257}.
/FTId=VAR_077302.
VARIANT 410 410 G -> R (in MCC2D).
{ECO:0000269|PubMed:27601257}.
/FTId=VAR_077303.
VARIANT 434 434 V -> L (in MCC2D; has some wild-type
residual activity; dbSNP:rs758506791).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072531.
VARIANT 437 437 I -> V (in MCC2D; mild form;
dbSNP:rs119103224).
/FTId=VAR_012801.
VARIANT 441 441 I -> T (in MCC2D; unknown pathological
significance; dbSNP:rs139852818).
{ECO:0000269|PubMed:27601257}.
/FTId=VAR_077304.
VARIANT 456 456 A -> V (in MCC2D; shows virtually no
enzyme activity; dbSNP:rs727504011).
{ECO:0000269|PubMed:16010683,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_072532.
VARIANT 459 459 P -> S (in MCC2D; dbSNP:rs754741111).
{ECO:0000269|PubMed:22150417}.
/FTId=VAR_067200.
VARIANT 461 461 F -> V (in MCC2D).
{ECO:0000269|PubMed:27601257}.
/FTId=VAR_077305.
VARIANT 475 475 G -> R (in MCC2D; has some wild-type
residual activity; dbSNP:rs148773718).
{ECO:0000269|PubMed:22642865,
ECO:0000269|PubMed:27601257}.
/FTId=VAR_072533.
VARIANT 477 477 Q -> R (in MCC2D; dbSNP:rs769558016).
{ECO:0000269|PubMed:21071250,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_072534.
VARIANT 478 478 A -> G (in dbSNP:rs35068278).
/FTId=VAR_038630.
VARIANT 517 517 G -> R (in MCC2D; dbSNP:rs979584886).
{ECO:0000269|PubMed:21071250,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_072535.
VARIANT 520 520 Y -> S (in MCC2D; dbSNP:rs150327768).
{ECO:0000269|PubMed:21071250,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_072536.
VARIANT 523 523 S -> G (in MCC2D; has some wild-type
residual activity; dbSNP:rs1459143051).
{ECO:0000269|PubMed:22264772,
ECO:0000269|PubMed:22642865}.
/FTId=VAR_072537.
VARIANT 524 524 A -> T (in MCC2D; dbSNP:rs774241918).
{ECO:0000269|PubMed:27601257}.
/FTId=VAR_077306.
VARIANT 555 555 K -> E (in MCC2D; dbSNP:rs1257849672).
{ECO:0000269|PubMed:22642865}.
/FTId=VAR_072538.
SEQUENCE 563 AA; 61333 MW; 8E3D401AF52DC7D2 CRC64;
MWAVLRLALR PCARASPAGP RAYHGDSVAS LGTQPDLGSA LYQENYKQMK ALVNQLHERV
EHIKLGGGEK ARALHISRGK LLPRERIDNL IDPGSPFLEL SQFAGYQLYD NEEVPGGGII
TGIGRVSGVE CMIIANDATV KGGAYYPVTV KKQLRAQEIA MQNRLPCIYL VDSGGAYLPR
QADVFPDRDH FGRTFYNQAI MSSKNIAQIA VVMGSCTAGG AYVPAMADEN IIVRKQGTIF
LAGPPLVKAA TGEEVSAEDL GGADLHCRKS GVSDHWALDD HHALHLTRKV VRNLNYQKKL
DVTIEPSEEP LFPADELYGI VGANLKRSFD VREVIARIVD GSRFTEFKAF YGDTLVTGFA
RIFGYPVGIV GNNGVLFSES AKKGTHFVQL CCQRNIPLLF LQNITGFMVG REYEAEGIAK
DGAKMVAAVA CAQVPKITLI IGGSYGAGNY GMCGRAYSPR FLYIWPNARI SVMGGEQAAN
VLATITKDQR AREGKQFSSA DEAALKEPII KKFEEEGNPY YSSARVWDDG IIDPADTRLV
LGLSFSAALN APIEKTDFGI FRM


Related products :

Catalog number Product name Quantity
EIAAB30046 PCCase subunit beta,PCCB,Pig,Propanoyl-CoA carbon dioxide ligase subunit beta,Propionyl-CoA carboxylase beta chain, mitochondrial,Sus scrofa
EIAAB30045 Bos taurus,Bovine,PCCase subunit beta,PCCB,Propanoyl-CoA carbon dioxide ligase subunit beta,Propionyl-CoA carboxylase beta chain, mitochondrial
EIAAB30044 PCCase subunit beta,Pccb,Propanoyl-CoA carbon dioxide ligase subunit beta,Propionyl-CoA carboxylase beta chain, mitochondrial,Rat,Rattus norvegicus
EIAAB30047 Mouse,Mus musculus,PCCase subunit beta,Pccb,Propanoyl-CoA carbon dioxide ligase subunit beta,Propionyl-CoA carboxylase beta chain, mitochondrial
EIAAB30043 Homo sapiens,Human,PCCase subunit beta,PCCB,Propanoyl-CoA carbon dioxide ligase subunit beta,Propionyl-CoA carboxylase beta chain, mitochondrial
15-288-21224 Propionyl-CoA carboxylase alpha chain. mitochondrial - EC 6.4.1.3; PCCase subunit alpha; Propanoyl-CoA carbon dioxide ligase subunit alpha Polyclonal 0.1 mg
15-288-21224 Propionyl-CoA carboxylase alpha chain. mitochondrial - EC 6.4.1.3; PCCase subunit alpha; Propanoyl-CoA carbon dioxide ligase subunit alpha Polyclonal 0.05 mg
EIAAB30042 Mouse,Mus musculus,Pcca,PCCase subunit alpha,Propanoyl-CoA carbon dioxide ligase subunit alpha,Propionyl-CoA carboxylase alpha chain, mitochondrial
EIAAB30041 Pcca,PCCase subunit alpha,Propanoyl-CoA carbon dioxide ligase subunit alpha,Propionyl-CoA carboxylase alpha chain, mitochondrial,Rat,Rattus norvegicus
EIAAB30040 Homo sapiens,Human,PCCA,PCCase subunit alpha,Propanoyl-CoA carbon dioxide ligase subunit alpha,Propionyl-CoA carboxylase alpha chain, mitochondrial
SPAG7_HUMAN Mouse ELISA Kit FOR Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial 96T
CSB-EL013573RA Rat Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial(MCCC2) ELISA kit 96T
E1570h Human ELISA Kit FOR Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial 96T
E0703r Mouse ELISA Kit FOR Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial 96T
CSB-EL013573MO Mouse Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial(MCCC2) ELISA kit 96T
CSB-EL013573RA Rat Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial(MCCC2) ELISA kit SpeciesRat 96T
CSB-EL013573HU Human Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial(MCCC2) ELISA kit 96T
CSB-EL013573MO Mouse Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial(MCCC2) ELISA kit SpeciesMouse 96T
CSB-EL013573HU Human Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial(MCCC2) ELISA kit SpeciesHuman 96T
MCCB_MOUSE ELISA Kit FOR Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial; organism: Mouse; gene name: Mccc2 96T
E0195h Human ELISA Kit FOR Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial 96T
STOX1_HUMAN Human ELISA Kit FOR Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial 96T
HCST_MOUSE Human ELISA Kit FOR Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial 96T
CSB-EL013572RA Rat Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial(MCCC1) ELISA kit 96T
CSB-EL013572RA Rat Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial(MCCC1) ELISA kit SpeciesRat 96T
Pathways :
WP1614: 1- and 2-Methylnaphthalene degradation
WP1655: Geraniol degradation
WP1566: Citrate cycle (TCA cycle)
WP296: TCA Cycle - biocyc
WP401: Mitochondrial LC-Fatty Acid Beta-Oxidation
WP419: Mitochondrial LC-Fatty Acid Beta-Oxidation
WP871: Mitochondrial LC-Fatty Acid Beta-Oxidation
WP1107: Mitochondrial LC-Fatty Acid Beta-Oxidation
WP1226: Mitochondrial LC-Fatty Acid Beta-Oxidation
WP763: Mitochondrial LC-Fatty Acid Beta-Oxidation
WP989: Mitochondrial LC-Fatty Acid Beta-Oxidation
WP368: Mitochondrial LC-Fatty Acid Beta-Oxidation
WP1626: Benzoate degradation via CoA ligation
WP1711: Trinitrotoluene degradation
WP1493: Carbon assimilation C4 pathway
WP448: Mitochondrial LC-Fatty Acid Beta-Oxidation
WP1672: Mismatch repair
WP498: Mitochondrial LC-Fatty Acid Beta-Oxidation
WP542: Electron Transport Chain
WP406: Mitochondrial LC-Fatty Acid Beta-Oxidation
WP1671: Methane metabolism
WP372: Beta Oxidation Meta Pathway
WP1048: TGF Beta Signaling Pathway
WP1307: Fatty Acid Beta Oxidation
WP505: TGF Beta Signaling Pathway

Related Genes :
[MCCC2 MCCB] Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCase subunit beta) (EC 6.4.1.4) (3-methylcrotonyl-CoA carboxylase 2) (3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit) (3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta)
[MCCC1 MCCA] Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (MCCase subunit alpha) (EC 6.4.1.4) (3-methylcrotonyl-CoA carboxylase 1) (3-methylcrotonyl-CoA carboxylase biotin-containing subunit) (3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha)
[PCCB] Propionyl-CoA carboxylase beta chain, mitochondrial (PCCase subunit beta) (EC 6.4.1.3) (Propanoyl-CoA:carbon dioxide ligase subunit beta)
[HLCS] Biotin--protein ligase (EC 6.3.4.-) (Biotin apo-protein ligase) [Includes: Biotin--[methylmalonyl-CoA-carboxytransferase] ligase (EC 6.3.4.9); Biotin--[propionyl-CoA-carboxylase [ATP-hydrolyzing]] ligase (EC 6.3.4.10) (Holocarboxylase synthetase) (HCS); Biotin--[methylcrotonoyl-CoA-carboxylase] ligase (EC 6.3.4.11); Biotin--[acetyl-CoA-carboxylase] ligase (EC 6.3.4.15)]
[Acacb Acc2 Accb] Acetyl-CoA carboxylase 2 (EC 6.4.1.2) (ACC-beta) [Includes: Biotin carboxylase (EC 6.3.4.14)]
[ACC1 ABP2 FAS3 MTR7 YNR016C N3175] Acetyl-CoA carboxylase (ACC) (EC 6.4.1.2) (Fatty acid synthetase 3) (mRNA transport-defective protein 7) [Includes: Biotin carboxylase (EC 6.3.4.14)]
[accD dedB usg b2316 JW2313] Acetyl-coenzyme A carboxylase carboxyl transferase subunit beta (ACCase subunit beta) (Acetyl-CoA carboxylase carboxyltransferase subunit beta) (EC 2.1.3.15)
[accD ycf11 zfpA] Acetyl-coenzyme A carboxylase carboxyl transferase subunit beta, chloroplastic (ACCase subunit beta) (Acetyl-CoA carboxylase carboxyltransferase subunit beta) (EC 2.1.3.15)
[birA bioR dhbB b3973 JW3941] Bifunctional ligase/repressor BirA (Biotin operon repressor) (Biotin--[acetyl-CoA-carboxylase] ligase) (EC 6.3.4.15) (Biotin--protein ligase) (Biotin-[acetyl-CoA carboxylase] synthetase)
[CAC2 At5g35360 T26D22.8] Biotin carboxylase, chloroplastic (EC 6.3.4.14) (Acetyl-CoA carboxylase subunit A) (ACC) (EC 6.4.1.2)
[ACC1 EMB22 GK PAS3 At1g36160 F15C21.1] Acetyl-CoA carboxylase 1 (AtACC1) (EC 6.4.1.2) (Protein EMBRYO DEFECTIVE 22) (Protein GURKE) (Protein PASTICCINO 3) [Includes: Biotin carboxylase (EC 6.3.4.14)]
[Acaca Acac] Acetyl-CoA carboxylase 1 (ACC1) (EC 6.4.1.2) (ACC-alpha) [Includes: Biotin carboxylase (EC 6.3.4.14)]
[accA b0185 JW0180] Acetyl-coenzyme A carboxylase carboxyl transferase subunit alpha (ACCase subunit alpha) (Acetyl-CoA carboxylase carboxyltransferase subunit alpha) (EC 2.1.3.15)
[HFA1 YMR207C YM8261.01C YM8325.08C] Acetyl-CoA carboxylase, mitochondrial (ACC) (EC 6.4.1.2) [Includes: Biotin carboxylase (EC 6.3.4.14)]
[SUCLA2] Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial (EC 6.2.1.5) (ATP-specific succinyl-CoA synthetase subunit beta) (A-SCS) (Succinyl-CoA synthetase beta-A chain) (SCS-betaA)
[] 4-chlorobenzoyl coenzyme A dehalogenase (4-CBA-CoA dehalogenase) (4-CBCoA dehalogenase) (4-chlorobenzoyl-CoA dehalogenase) (EC 3.8.1.7)
[Prkaa1] 5'-AMP-activated protein kinase catalytic subunit alpha-1 (AMPK subunit alpha-1) (EC 2.7.11.1) (Acetyl-CoA carboxylase kinase) (ACACA kinase) (EC 2.7.11.27) (Hydroxymethylglutaryl-CoA reductase kinase) (HMGCR kinase) (EC 2.7.11.31) (Tau-protein kinase PRKAA1) (EC 2.7.11.26)
[Prkaa2] 5'-AMP-activated protein kinase catalytic subunit alpha-2 (AMPK subunit alpha-2) (EC 2.7.11.1) (Acetyl-CoA carboxylase kinase) (ACACA kinase) (EC 2.7.11.27) (Hydroxymethylglutaryl-CoA reductase kinase) (HMGCR kinase) (EC 2.7.11.31)
[accD AtCg00500] Acetyl-coenzyme A carboxylase carboxyl transferase subunit beta, chloroplastic (ACCase subunit beta) (Acetyl-CoA carboxylase carboxyltransferase subunit beta) (EC 2.1.3.15)
[Prkaa2 Ampk Ampk2] 5'-AMP-activated protein kinase catalytic subunit alpha-2 (AMPK subunit alpha-2) (EC 2.7.11.1) (Acetyl-CoA carboxylase kinase) (ACACA kinase) (EC 2.7.11.27) (Hydroxymethylglutaryl-CoA reductase kinase) (HMGCR kinase) (EC 2.7.11.31)
[HSD17B10 ERAB HADH2 MRPP2 SCHAD SDR5C1 XH98G2] 3-hydroxyacyl-CoA dehydrogenase type-2 (EC 1.1.1.35) (17-beta-hydroxysteroid dehydrogenase 10) (17-beta-HSD 10) (EC 1.1.1.51) (2-methyl-3-hydroxybutyryl-CoA dehydrogenase) (MHBD) (3-hydroxy-2-methylbutyryl-CoA dehydrogenase) (EC 1.1.1.178) (3-hydroxyacyl-CoA dehydrogenase type II) (Endoplasmic reticulum-associated amyloid beta-peptide-binding protein) (Mitochondrial ribonuclease P protein 2) (Mitochondrial RNase P protein 2) (Short chain dehydrogenase/reductase family 5C member 1) (Short-chain type dehydrogenase/reductase XH98G2) (Type II HADH)
[Prkaa1 Ampk1] 5'-AMP-activated protein kinase catalytic subunit alpha-1 (AMPK subunit alpha-1) (EC 2.7.11.1) (Acetyl-CoA carboxylase kinase) (ACACA kinase) (EC 2.7.11.27) (Hydroxymethylglutaryl-CoA reductase kinase) (HMGCR kinase) (EC 2.7.11.31) (Tau-protein kinase PRKAA1) (EC 2.7.11.26)
[Echs1] Enoyl-CoA hydratase, mitochondrial (EC 4.2.1.17) (Enoyl-CoA hydratase 1) (Short-chain enoyl-CoA hydratase) (SCEH)
[PRKAA1 AMPK1] 5'-AMP-activated protein kinase catalytic subunit alpha-1 (AMPK subunit alpha-1) (EC 2.7.11.1) (Acetyl-CoA carboxylase kinase) (ACACA kinase) (EC 2.7.11.27) (Hydroxymethylglutaryl-CoA reductase kinase) (HMGCR kinase) (EC 2.7.11.31) (Tau-protein kinase PRKAA1) (EC 2.7.11.26)
[mcl1 RHOS4_03500 RSP_1771] L-malyl-CoA/beta-methylmalyl-CoA lyase (EC 4.1.3.24) ((3S)-malyl-CoA/beta-methylmalyl-CoA lyase) ((S)-citramalyl-CoA lyase) (EC 4.1.3.25)
[PRKAA1 AMPK1] 5'-AMP-activated protein kinase catalytic subunit alpha-1 (AMPK subunit alpha-1) (EC 2.7.11.1) (AMPK 63 kDa subunit) (Acetyl-CoA carboxylase kinase) (ACACA kinase) (EC 2.7.11.27) (Hydroxymethylglutaryl-CoA reductase kinase) (HMGCR kinase) (EC 2.7.11.31) (Tau-protein kinase PRKAA1) (EC 2.7.11.26) (Fragments)
[PRKAA2 AMPK AMPK2] 5'-AMP-activated protein kinase catalytic subunit alpha-2 (AMPK subunit alpha-2) (EC 2.7.11.1) (Acetyl-CoA carboxylase kinase) (ACACA kinase) (EC 2.7.11.27) (Hydroxymethylglutaryl-CoA reductase kinase) (HMGCR kinase) (EC 2.7.11.31)
[PRKAA1] 5'-AMP-activated protein kinase catalytic subunit alpha-1 (AMPK subunit alpha-1) (EC 2.7.11.1) (Acetyl-CoA carboxylase kinase) (ACACA kinase) (EC 2.7.11.27) (Hydroxymethylglutaryl-CoA reductase kinase) (HMGCR kinase) (EC 2.7.11.31) (Tau-protein kinase PRKAA1) (EC 2.7.11.26) (Fragment)
[Hsd17b10 Erab Hadh2] 3-hydroxyacyl-CoA dehydrogenase type-2 (EC 1.1.1.35) (17-beta-hydroxysteroid dehydrogenase 10) (17-beta-HSD 10) (EC 1.1.1.51) (3-hydroxy-2-methylbutyryl-CoA dehydrogenase) (EC 1.1.1.178) (3-hydroxyacyl-CoA dehydrogenase type II) (Endoplasmic reticulum-associated amyloid beta-peptide-binding protein) (Mitochondrial ribonuclease P protein 2) (Mitochondrial RNase P protein 2) (Type II HADH)
[scsA DDB_G0289325] Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial (EC 6.2.1.4) (EC 6.2.1.5) (Succinyl-CoA synthetase subunit alpha) (SCS-alpha) (p36)

Bibliography :
No related Items