8XL8

Structure of human 3-methylcrotonyl-CoA carboxylase in complex with propionyl-CoA (MCC-PCO)

External Resource: Annotation

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

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A, C, E, G, I A, C, E, G, I, K	PF00364	Biotin-requiring enzyme (Biotin_lipoyl)	Biotin-requiring enzyme	This family covers two Prosite entries, the conserved lysine residue binds biotin in one group and lipoic acid in the other. Note that the HMM does not currently recognise the Glycine cleavage system H proteins.	Domain
A, C, E, G, I A, C, E, G, I, K	PF02786	Carbamoyl-phosphate synthase L chain, ATP binding domain (CPSase_L_D2)	Carbamoyl-phosphate synthase L chain, ATP binding domain	Carbamoyl-phosphate synthase catalyses the ATP-dependent synthesis of carbamyl-phosphate from glutamine or ammonia and bicarbonate. This important enzyme initiates both the urea cycle and the biosynthesis of arginine and/or pyrimidines [2]. The c ...	Carbamoyl-phosphate synthase catalyses the ATP-dependent synthesis of carbamyl-phosphate from glutamine or ammonia and bicarbonate. This important enzyme initiates both the urea cycle and the biosynthesis of arginine and/or pyrimidines [2]. The carbamoyl-phosphate synthase (CPS) enzyme in prokaryotes is a heterodimer of a small and large chain. The small chain promotes the hydrolysis of glutamine to ammonia, which is used by the large chain to synthesise carbamoyl phosphate. See Pfam:PF00988. The small chain has a GATase domain in the carboxyl terminus. See Pfam:PF00117. The ATP binding domain (this one) has an ATP-grasp fold.	Domain
A, C, E, G, I A, C, E, G, I, K	PF02785	Biotin carboxylase C-terminal domain (Biotin_carb_C)	Biotin carboxylase C-terminal domain	Biotin carboxylase is a component of the acetyl-CoA carboxylase multi-component enzyme which catalyses the first committed step in fatty acid synthesis in animals, plants and bacteria. Most of the active site residues reported in reference [1] are i ...	Biotin carboxylase is a component of the acetyl-CoA carboxylase multi-component enzyme which catalyses the first committed step in fatty acid synthesis in animals, plants and bacteria. Most of the active site residues reported in reference [1] are in this C-terminal domain.	Domain
B, D, F, H, J B, D, F, H, J, L	PF01039	Carboxyl transferase domain (Carboxyl_trans)	Carboxyl transferase domain	-	Family

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A, C, E, G, I A, C, E, G, I, K	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	catabolic process oxoacid 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 carboxylic acid catabolic process small molecule metabolic process metabolic process cellular process branched-chain amino acid metabolic process catabolic process oxoacid 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 carboxylic acid catabolic process small molecule metabolic process metabolic process cellular process branched-chain amino acid metabolic process small molecule catabolic process amino acid catabolic process biotin metabolic process vitamin 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 cellular anatomical structure intracellular anatomical structure mitochondrion organelle cytoplasm intracellular membrane-bounded organelle cytosol protein-containing complex catalytic complex methylcrotonoyl-CoA carboxylase complex membrane-enclosed lumen mitochondrial matrix membrane-bounded organelle intracellular organelle cellular anatomical structure intracellular anatomical structure mitochondrion organelle cytoplasm intracellular membrane-bounded organelle cytosol protein-containing complex catalytic complex methylcrotonoyl-CoA carboxylase complex membrane-enclosed lumen mitochondrial matrix intracellular organelle lumen organelle lumen
B, D, F, H, J B, D, F, H, J, L	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	catabolic process oxoacid 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 carboxylic acid catabolic process small molecule metabolic process metabolic process cellular process branched-chain amino acid metabolic process catabolic process oxoacid 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 carboxylic acid catabolic process small molecule metabolic process metabolic process cellular process branched-chain amino acid metabolic process small molecule catabolic process amino acid catabolic process nucleoside phosphate metabolic process purine-containing compound metabolic process nucleobase-containing compound metabolic process organophosphate metabolic process nucleobase-containing small molecule metabolic process phosphate-containing compound metabolic process sulfur compound metabolic process amide metabolic process phosphorus metabolic process coenzyme A 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 cellular anatomical structure intracellular anatomical structure mitochondrion organelle cytoplasm intracellular membrane-bounded organelle cytosol protein-containing complex catalytic complex methylcrotonoyl-CoA carboxylase complex membrane-enclosed lumen mitochondrial matrix membrane-bounded organelle intracellular organelle cellular anatomical structure intracellular anatomical structure mitochondrion organelle cytoplasm intracellular membrane-bounded organelle cytosol protein-containing complex catalytic complex methylcrotonoyl-CoA carboxylase complex membrane-enclosed lumen mitochondrial matrix intracellular organelle lumen organelle lumen

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A, C, E, G, I A, C, E, G, I, K	IPR005481	Biotin carboxylase-like, N-terminal domain	Domain
A, C, E, G, I A, C, E, G, I, K	IPR011761	ATP-grasp fold	Domain
A, C, E, G, I A, C, E, G, I, K	IPR011764	Biotin carboxylation domain	Domain
A, C, E, G, I A, C, E, G, I, K	IPR050856	Biotin-dependent Carboxylase Complex	Family
A, C, E, G, I A, C, E, G, I, K	IPR005482	Biotin carboxylase, C-terminal	Domain
A, C, E, G, I A, C, E, G, I, K	IPR005479	Carbamoyl-phosphate synthetase large subunit-like, ATP-binding domain	Domain
A, C, E, G, I A, C, E, G, I, K	IPR016185	Pre-ATP-grasp domain superfamily	Homologous Superfamily
A, C, E, G, I A, C, E, G, I, K	IPR011054	Rudiment single hybrid motif	Homologous Superfamily
A, C, E, G, I A, C, E, G, I, K	IPR011053	Single hybrid motif	Homologous Superfamily
A, C, E, G, I A, C, E, G, I, K	IPR001882	Biotin-binding site	Binding Site
A, C, E, G, I A, C, E, G, I, K	IPR000089	Biotin/lipoyl attachment	Domain
B, D, F, H, J B, D, F, H, J, L	IPR011763	Acetyl-coenzyme A carboxyltransferase, C-terminal	Domain
B, D, F, H, J B, D, F, H, J, L	IPR029045	ClpP/crotonase-like domain superfamily	Homologous Superfamily
B, D, F, H, J B, D, F, H, J, L	IPR011762	Acetyl-coenzyme A carboxyltransferase, N-terminal	Domain
B, D, F, H, J B, D, F, H, J, L	IPR034733	Acetyl-coenzyme A carboxylase carboxyl transferase subunit beta	Domain
B, D, F, H, J B, D, F, H, J, L	IPR045190	Methylcrotonoyl-CoA carboxylase beta chain MCCB/AccD1-like	Family

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, C, E, G, I A, C, E, G, I, K	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
B, D, F, H, J B, D, F, H, J, L	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

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 R01GM157729.