GlyCosmos Portal

Submissions

methylcrotonyl-CoA carboxylase subunit 1

Summary

Gene Symbol

MCCC1

Organism

Homo sapiens (human)

External Links

NCBI Gene

56922

PubChem

56922

Alliance of Genome Resources

HGNC:6936

Annotation

Keyword

3D-structure
ATP-binding
Acetylation
Biotin
Direct protein sequencing
Disease variant
Ligase
Mitochondrion
Reference proteome
Transit peptide

Proteins

Displaying **all 3** entries
UniProt	Protein Name
Q96RQ3	3-methylcrotonyl-CoA carboxylase 1 3-methylcrotonyl-CoA carboxylase biotin-containing subunit 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha
Q68D27
A0A0S2Z693

Annotation information from UniProt.

Gene Ontology (GO)

Displaying **all 5** entries
GO Term	Evidence Code	PMID
leucine catabolic process	ISS	11170888
leucine catabolic process	NAS	11170888
leucine catabolic process	IEA
biotin metabolic process	NAS	11401427 22869039
branched-chain amino acid catabolic process	NAS	17360195

GO Hierarchy

biological process

metabolic process [inference]

nitrogen compound metabolic process [inference]

amide metabolic process [inference]

biotin metabolic process

organonitrogen compound metabolic process [inference]

amino acid metabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

branched-chain amino acid metabolic process [inference]

leucine metabolic process [inference]

leucine catabolic process

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid metabolic process [inference]

leucine metabolic process [inference]

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

biotin metabolic process

organonitrogen compound catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

catabolic process [inference]

cellular catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

small molecule catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

organic substance catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

organonitrogen compound catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

cellular metabolic process [inference]

organic acid metabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

amino acid metabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

branched-chain amino acid metabolic process [inference]

leucine metabolic process [inference]

leucine catabolic process

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid metabolic process [inference]

leucine metabolic process [inference]

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

monocarboxylic acid metabolic process [inference]

biotin metabolic process

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

sulfur compound metabolic process [inference]

biotin metabolic process

cellular catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

heterocycle metabolic process [inference]

biotin metabolic process

primary metabolic process [inference]

amino acid metabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

branched-chain amino acid metabolic process [inference]

leucine metabolic process [inference]

leucine catabolic process

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid metabolic process [inference]

leucine metabolic process [inference]

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

small molecule metabolic process [inference]

organic acid metabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

amino acid metabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

branched-chain amino acid metabolic process [inference]

leucine metabolic process [inference]

leucine catabolic process

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid metabolic process [inference]

leucine metabolic process [inference]

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

monocarboxylic acid metabolic process [inference]

biotin metabolic process

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

vitamin metabolic process [inference]

water-soluble vitamin metabolic process [inference]

biotin metabolic process

small molecule catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

organic substance metabolic process [inference]

organic acid metabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

amino acid metabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

branched-chain amino acid metabolic process [inference]

leucine metabolic process [inference]

leucine catabolic process

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid metabolic process [inference]

leucine metabolic process [inference]

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

monocarboxylic acid metabolic process [inference]

biotin metabolic process

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

organic cyclic compound metabolic process [inference]

biotin metabolic process

organonitrogen compound metabolic process [inference]

amino acid metabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

branched-chain amino acid metabolic process [inference]

leucine metabolic process [inference]

leucine catabolic process

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid metabolic process [inference]

leucine metabolic process [inference]

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

biotin metabolic process

organonitrogen compound catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

organic substance catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

organonitrogen compound catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

cellular process [inference]

cellular metabolic process [inference]

organic acid metabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

amino acid metabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

branched-chain amino acid metabolic process [inference]

leucine metabolic process [inference]

leucine catabolic process

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid metabolic process [inference]

leucine metabolic process [inference]

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

monocarboxylic acid metabolic process [inference]

biotin metabolic process

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

sulfur compound metabolic process [inference]

biotin metabolic process

cellular catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

leucine catabolic process

alpha-amino acid catabolic process [inference]

leucine catabolic process

heterocycle metabolic process [inference]

biotin metabolic process

Displaying entries **1 - 5** of 12 in total

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GO Term	Evidence Code	PMID
3-methylcrotonyl-CoA carboxylase complex, mitochondrial
mitochondrion	IDA
mitochondrion	IBA
mitochondrion	HTP	34800366
mitochondrial matrix	NAS	11170888

GO Hierarchy

cellular component

protein-containing complex [inference]

mitochondrial protein-containing complex [inference]

3-methylcrotonyl-CoA carboxylase complex, mitochondrial

catalytic complex [inference]

methylcrotonoyl-CoA carboxylase complex

3-methylcrotonyl-CoA carboxylase complex, mitochondrial

cellular anatomical entity [inference]

cytosol

membrane-enclosed lumen [inference]

organelle lumen [inference]

intracellular organelle lumen [inference]

mitochondrial matrix

organelle [inference]

membrane-bounded organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

Displaying entries **1 - 5** of 8 in total

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GO Term	Evidence Code	PMID
biotin carboxylase activity	NAS	22869039
methylcrotonoyl-CoA carboxylase activity	NAS	11170888 11181649 11401427
methylcrotonoyl-CoA carboxylase activity	IDA	17360195
methylcrotonoyl-CoA carboxylase activity	IBA
protein binding	IPI	17360195 27499296 28514442 33961781

GO Hierarchy

molecular function

catalytic activity [inference]

ligase activity [inference]

ligase activity, forming carbon-nitrogen bonds [inference]

biotin carboxylase activity

ligase activity, forming carbon-carbon bonds [inference]

CoA carboxylase activity [inference]

methylcrotonoyl-CoA carboxylase activity

binding [inference]

protein binding

amide binding [inference]

biotin binding

small molecule binding [inference]

vitamin binding [inference]

biotin binding

organic acid binding [inference]

carboxylic acid binding [inference]

monocarboxylic acid binding [inference]

biotin binding

heterocyclic compound binding [inference]

nucleotide binding [inference]

purine nucleotide binding [inference]

adenyl nucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

biotin binding

ion binding [inference]

anion binding [inference]

carboxylic acid binding [inference]

monocarboxylic acid binding [inference]

biotin binding

purine ribonucleoside triphosphate binding [inference]

ATP binding

cation binding [inference]

metal ion binding

organic cyclic compound binding [inference]

biotin binding

nucleoside phosphate binding [inference]

nucleotide binding [inference]

purine nucleotide binding [inference]

adenyl nucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

purine ribonucleoside triphosphate binding [inference]

ATP binding

carbohydrate derivative binding [inference]

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

sulfur compound binding [inference]

biotin binding

Gene Ontology information from NCBI Gene and UniProt.

OrthoDB (Group)

Group level

Eukaryota

Group Name

carboxylase

Functional Category

C: Energy production and conversion
E: Amino acid transport and metabolism
I: Lipid transport and metabolism
Q: Secondary metabolites biosynthesis, transport and catabolism

Displaying **all 7** entries
Gene Ontology
ATP binding
ligase activity
metal ion binding
methylcrotonoyl-CoA carboxylase activity
nucleotide binding
propionyl-CoA carboxylase activity
urea carboxylase activity

Displaying **all 9** entries
InterPro
ATP-grasp fold
Biotin carboxylase, C-terminal
Biotin carboxylase-like, N-terminal domain
Biotin carboxylation domain
Biotin/lipoyl attachment
Carbamoyl-phosphate synthetase large subunit-like, ATP-binding domain
Pre-ATP-grasp domain superfamily
Rudiment single hybrid motif
Single hybrid motif

Disease

Disease Ontology

Displaying 1 entry
DO ID	Disease Name	Source
DOID:0080579	3-methylcrotonyl-CoA carboxylase 1 deficiency	Alliance of Genome Resources

Ortholog

Displaying entries **1 - 10** of 80 in total

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Species	Gene ID	Alliance of Genome Resources	Orthologous MAtrix
Xenopus tropicalis	56922	Xenbase:XB-GENE-967301
Mus musculus	72039	Xenbase:XB-GENE-967301	MOUSE36768
Rattus norvegicus	294972		RATNO24813
Pan troglodytes	460872		PANTR32749
Canis lupus familiaris	478645		CANLF13507
Bos taurus	513504		BOVIN14917
Macaca mulatta	708258		MACMU28230
Danio rerio	777632		DANRE22746
Monodelphis domestica	100016006		MONDO31090
Equus caballus	100058574		HORSE14924

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OrthoDB (Group)

International Collaboration

Acknowledgements