8J4Z

Human 3-methylcrotonyl-CoA carboxylase in BCCP-CTS state with substrate

External Resource: Annotation

Gene Ontology: Gene Product Annotation
InterPro: Protein Family Classification
Pharos: Disease Associations

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A [auth J], C [auth A], E [auth C], G [auth F], I [auth H], A [auth J], C [auth A], E [auth C], G [auth F], I [auth H], K	Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial	binding adenyl ribonucleotide binding nucleotide binding purine ribonucleotide binding purine ribonucleoside triphosphate binding nucleoside phosphate binding ion binding purine nucleotide binding organic cyclic compound binding anion binding adenyl nucleotide binding ATP binding heterocyclic compound binding carbohydrate derivative binding binding adenyl ribonucleotide binding nucleotide binding purine ribonucleotide binding purine ribonucleoside triphosphate binding nucleoside phosphate binding ion binding purine nucleotide binding organic cyclic compound binding anion binding adenyl nucleotide binding ATP binding heterocyclic compound binding carbohydrate derivative binding ribonucleotide binding small molecule binding CoA carboxylase activity methylcrotonoyl-CoA carboxylase activity ligase activity, forming carbon-carbon bonds catalytic activity ligase activity	organonitrogen compound catabolic process catabolic process organonitrogen compound metabolic process oxoacid metabolic process cellular metabolic process carboxylic acid metabolic process amino acid metabolic process primary metabolic process organic acid catabolic process organic acid metabolic process branched-chain amino acid catabolic process cellular catabolic process organic substance metabolic process carboxylic acid catabolic process organonitrogen compound catabolic process catabolic process organonitrogen compound metabolic process oxoacid metabolic process cellular metabolic process carboxylic acid metabolic process amino acid metabolic process primary metabolic process organic acid catabolic process organic acid metabolic process branched-chain amino acid catabolic process cellular catabolic process organic substance metabolic process carboxylic acid catabolic process small molecule metabolic process metabolic process cellular process branched-chain amino acid metabolic process small molecule catabolic process organic substance catabolic process amino acid catabolic process ribose phosphate metabolic process nucleoside bisphosphate metabolic process purine nucleoside bisphosphate metabolic process ribonucleoside bisphosphate metabolic process carbohydrate derivative metabolic process nucleobase-containing compound metabolic process purine nucleotide metabolic process ribonucleotide metabolic process nucleobase-containing small molecule metabolic process phosphate-containing compound metabolic process phosphorus metabolic process coenzyme A metabolic process nucleoside phosphate metabolic process nucleotide metabolic process purine-containing compound metabolic process organic cyclic compound metabolic process organophosphate metabolic process purine ribonucleotide metabolic process pyruvate family amino acid metabolic process alpha-amino acid metabolic process pyruvate family amino acid catabolic process L-leucine metabolic process L-leucine catabolic process L-amino acid catabolic process L-amino acid metabolic process proteinogenic amino acid metabolic process alpha-amino acid catabolic process proteinogenic amino acid catabolic process	membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle cytosol protein-containing complex catalytic complex methylcrotonoyl-CoA carboxylase complex mitochondrial protein-containing complex 3-methylcrotonyl-CoA carboxylase complex, mitochondrial membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle cytosol protein-containing complex catalytic complex methylcrotonoyl-CoA carboxylase complex mitochondrial protein-containing complex 3-methylcrotonyl-CoA carboxylase complex, mitochondrial membrane-enclosed lumen mitochondrial matrix intracellular organelle lumen organelle lumen
B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], L	Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial	binding adenyl ribonucleotide binding nucleotide binding purine ribonucleotide binding purine ribonucleoside triphosphate binding nucleoside phosphate binding ion binding purine nucleotide binding organic cyclic compound binding anion binding adenyl nucleotide binding ATP binding heterocyclic compound binding carbohydrate derivative binding binding adenyl ribonucleotide binding nucleotide binding purine ribonucleotide binding purine ribonucleoside triphosphate binding nucleoside phosphate binding ion binding purine nucleotide binding organic cyclic compound binding anion binding adenyl nucleotide binding ATP binding heterocyclic compound binding carbohydrate derivative binding ribonucleotide binding small molecule binding biotin binding amide binding organic acid binding vitamin binding carboxylic acid binding monocarboxylic acid binding sulfur compound binding biotin carboxylase activity catalytic activity ligase activity, forming carbon-nitrogen bonds ligase activity CoA carboxylase activity methylcrotonoyl-CoA carboxylase activity ligase activity, forming carbon-carbon bonds cation binding metal ion binding	organonitrogen compound catabolic process catabolic process organonitrogen compound metabolic process oxoacid metabolic process cellular metabolic process carboxylic acid metabolic process amino acid metabolic process primary metabolic process organic acid catabolic process organic acid metabolic process branched-chain amino acid catabolic process cellular catabolic process organic substance metabolic process carboxylic acid catabolic process organonitrogen compound catabolic process catabolic process organonitrogen compound metabolic process oxoacid metabolic process cellular metabolic process carboxylic acid metabolic process amino acid metabolic process primary metabolic process organic acid catabolic process organic acid metabolic process branched-chain amino acid catabolic process cellular catabolic process organic substance metabolic process carboxylic acid catabolic process small molecule metabolic process metabolic process cellular process branched-chain amino acid metabolic process small molecule catabolic process organic substance catabolic process amino acid catabolic process biotin metabolic process vitamin metabolic process organic cyclic compound metabolic process sulfur compound metabolic process amide metabolic process monocarboxylic acid metabolic process water-soluble vitamin metabolic process pyruvate family amino acid metabolic process alpha-amino acid metabolic process pyruvate family amino acid catabolic process L-leucine metabolic process L-leucine catabolic process L-amino acid catabolic process L-amino acid metabolic process proteinogenic amino acid metabolic process alpha-amino acid catabolic process proteinogenic amino acid catabolic process	membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle cytosol protein-containing complex catalytic complex methylcrotonoyl-CoA carboxylase complex mitochondrial protein-containing complex 3-methylcrotonyl-CoA carboxylase complex, mitochondrial membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle cytosol protein-containing complex catalytic complex methylcrotonoyl-CoA carboxylase complex mitochondrial protein-containing complex 3-methylcrotonyl-CoA carboxylase complex, mitochondrial membrane-enclosed lumen mitochondrial matrix intracellular organelle lumen organelle lumen

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A [auth J], C [auth A], E [auth C], G [auth F], I [auth H], A [auth J], C [auth A], E [auth C], G [auth F], I [auth H], K	IPR011763	Acetyl-coenzyme A carboxyltransferase, C-terminal	Domain
A [auth J], C [auth A], E [auth C], G [auth F], I [auth H], A [auth J], C [auth A], E [auth C], G [auth F], I [auth H], K	IPR029045	ClpP/crotonase-like domain superfamily	Homologous Superfamily
A [auth J], C [auth A], E [auth C], G [auth F], I [auth H], A [auth J], C [auth A], E [auth C], G [auth F], I [auth H], K	IPR011762	Acetyl-coenzyme A carboxyltransferase, N-terminal	Domain
A [auth J], C [auth A], E [auth C], G [auth F], I [auth H], A [auth J], C [auth A], E [auth C], G [auth F], I [auth H], K	IPR034733	Acetyl-coenzyme A carboxylase carboxyl transferase subunit beta	Domain
A [auth J], C [auth A], E [auth C], G [auth F], I [auth H], A [auth J], C [auth A], E [auth C], G [auth F], I [auth H], K	IPR045190	Methylcrotonoyl-CoA carboxylase beta chain MCCB/AccD1-like	Family
B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], L	IPR005481	Biotin carboxylase-like, N-terminal domain	Domain
B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], L	IPR011761	ATP-grasp fold	Domain
B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], L	IPR011764	Biotin carboxylation domain	Domain
B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], L	IPR005482	Biotin carboxylase, C-terminal	Domain
B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], L	IPR005479	Carbamoyl-phosphate synthetase large subunit-like, ATP-binding domain	Domain
B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], L	IPR016185	Pre-ATP-grasp domain superfamily	Homologous Superfamily
B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], L	IPR011054	Rudiment single hybrid motif	Homologous Superfamily
B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], L	IPR011053	Single hybrid motif	Homologous Superfamily
B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], L	IPR001882	Biotin-binding site	Binding Site
B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], L	IPR000089	Biotin/lipoyl attachment	Domain

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A [auth J], C [auth A], E [auth C], G [auth F], I [auth H], A [auth J], C [auth A], E [auth C], G [auth F], I [auth H], K	Pharos: Q9HCC0	3-methylcrotonyl-CoA carboxylase deficiency 3-methylcrotonyl-CoA carboxylase 2 deficiency pancreatic intraductal papillary-mucinous neoplasm 3-methylcrotonyl CoA carboxylase 2 deficiency breast cancer interstitial cystitis lung cancer osteosarcoma psoriasis 3-methylcrotonyl-CoA carboxylase 1 deficiency astrocytic tumor breast carcinoma inborn errors of metabolism invasive ductal breast carcinoma malignant ependymoma 3-methylcrotonyl-CoA carboxylase deficiency 3-methylcrotonyl-CoA carboxylase 2 deficiency pancreatic intraductal papillary-mucinous neoplasm 3-methylcrotonyl CoA carboxylase 2 deficiency breast cancer interstitial cystitis lung cancer osteosarcoma psoriasis 3-methylcrotonyl-CoA carboxylase 1 deficiency astrocytic tumor breast carcinoma inborn errors of metabolism invasive ductal breast carcinoma malignant ependymoma pancreatic ductal adenocarcinoma atypical teratoid rhabdoid tumor glioblastoma intellectual disability large cell medulloblastoma non-small cell lung carcinoma
B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], B [auth E], D [auth B], F [auth D], H [auth G], J [auth I], L	Pharos: Q96RQ3	3-methylcrotonyl-CoA carboxylase deficiency 3-methylcrotonyl-CoA carboxylase 1 deficiency Hyperammonemia interstitial cystitis osteosarcoma inborn errors of metabolism lung adenocarcinoma pancreatic ductal adenocarcinoma drug-induced liver injury intellectual disability malignant pancreatic neoplasm

RCSB PDB Core Operations are funded by the U.S. National Science Foundation (DBI-2321666), the US Department of Energy (DE-SC0019749), and the National Cancer Institute, National Institute of Allergy and Infectious Diseases, and National Institute of General Medical Sciences of the National Institutes of Health under grant R01GM133198.