8XL5

Structure of human propionyl-CoA carboxylase in complex with propionyl-CoA (PCC-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	PF18140	Propionyl-coenzyme A carboxylase BT domain (PCC_BT)	Propionyl-coenzyme A carboxylase BT domain	This domain is found in Propionyl-coenzyme A carboxylase (PCC), present in Roseobacter denitrificans. PCC is a mitochondrial biotin-dependent enzyme that is essential for the catabolism of certain amino acids, cholesterol, and fatty acids with an odd ...	This domain is found in Propionyl-coenzyme A carboxylase (PCC), present in Roseobacter denitrificans. PCC is a mitochondrial biotin-dependent enzyme that is essential for the catabolism of certain amino acids, cholesterol, and fatty acids with an odd number of carbon atoms. Since this domain mediates biotin carboxylase-carboxyltransferase interactions it is referred to as the BT domain. The BT domain is located between biotin carboxylase and the biotin carboxyl carrier protein domains. The BT domain shares some structural similarity with the pyruvate carboxylase tetramerization domain of pyruvate carboxylase [1].	Domain
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
A, C, E, G, I A, C, E, G, I, K	PF00289	Biotin carboxylase, N-terminal domain (Biotin_carb_N)	Biotin carboxylase, N-terminal domain	This domain is structurally related to the PreATP-grasp domain. The family contains the N-terminus of biotin carboxylase enzymes [1,3], and propionyl-CoA carboxylase A chain [2].	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	Propionyl-CoA carboxylase alpha chain, mitochondrial	CoA carboxylase activity propionyl-CoA carboxylase activity ligase activity, forming carbon-carbon bonds catalytic activity ligase activity 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 CoA carboxylase activity propionyl-CoA carboxylase activity ligase activity, forming carbon-carbon bonds catalytic activity ligase activity 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 protein binding enzyme binding cation binding metal ion binding	oxoacid metabolic process carboxylic acid metabolic process branched-chain amino acid metabolic process amino acid metabolic process primary metabolic process metabolic process cellular process small molecule metabolic process organic acid metabolic process fatty acid metabolic process monocarboxylic acid metabolic process lipid metabolic process short-chain fatty acid catabolic process lipid catabolic process oxoacid metabolic process carboxylic acid metabolic process branched-chain amino acid metabolic process amino acid metabolic process primary metabolic process metabolic process cellular process small molecule metabolic process organic acid metabolic process fatty acid metabolic process monocarboxylic acid metabolic process lipid metabolic process short-chain fatty acid catabolic process lipid catabolic process catabolic process short-chain fatty acid metabolic process organic acid catabolic process fatty acid catabolic process cellular catabolic process monocarboxylic acid catabolic process carboxylic acid catabolic process small molecule 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 membrane-enclosed lumen mitochondrial matrix intracellular organelle lumen membrane-bounded organelle intracellular organelle cellular anatomical structure intracellular anatomical structure mitochondrion organelle cytoplasm intracellular membrane-bounded organelle cytosol protein-containing complex catalytic complex membrane-enclosed lumen mitochondrial matrix intracellular organelle lumen organelle lumen
B, D, F, H, J B, D, F, H, J, L	Propionyl-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 propionyl-CoA carboxylase activity ligase activity, forming carbon-carbon bonds catalytic activity ligase activity	oxoacid metabolic process carboxylic acid metabolic process branched-chain amino acid metabolic process amino acid metabolic process primary metabolic process metabolic process cellular process small molecule metabolic process organic acid metabolic process fatty acid metabolic process monocarboxylic acid metabolic process lipid metabolic process short-chain fatty acid catabolic process lipid catabolic process oxoacid metabolic process carboxylic acid metabolic process branched-chain amino acid metabolic process amino acid metabolic process primary metabolic process metabolic process cellular process small molecule metabolic process organic acid metabolic process fatty acid metabolic process monocarboxylic acid metabolic process lipid metabolic process short-chain fatty acid catabolic process lipid catabolic process catabolic process short-chain fatty acid metabolic process organic acid catabolic process fatty acid catabolic process cellular catabolic process monocarboxylic acid catabolic process carboxylic acid catabolic process small molecule 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 membrane-enclosed lumen mitochondrial matrix intracellular organelle lumen membrane-bounded organelle intracellular organelle cellular anatomical structure intracellular anatomical structure mitochondrion organelle cytoplasm intracellular membrane-bounded organelle cytosol protein-containing complex catalytic 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	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	IPR011054	Rudiment single hybrid motif	Homologous Superfamily
A, C, E, G, I A, C, E, G, I, K	IPR041265	Propionyl-coenzyme A carboxylase, BT domain	Domain
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
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	IPR013815	ATP-grasp fold, subdomain 1	Homologous Superfamily
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
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	IPR051047	Acyl-CoA Carboxylase Beta Subunit	Family

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, C, E, G, I A, C, E, G, I, K	Pharos: P05165	propionic acidemia adrenal cortex carcinoma Epileptic encephalopathy inborn errors of metabolism interstitial cystitis osteosarcoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia attention deficit hyperactivity disorder, inattentive type glioblastoma inherited organic acidemia ovarian cancer tuberculosis breast cancer intellectual disability lung cancer propionic acidemia adrenal cortex carcinoma Epileptic encephalopathy inborn errors of metabolism interstitial cystitis osteosarcoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia attention deficit hyperactivity disorder, inattentive type glioblastoma inherited organic acidemia ovarian cancer tuberculosis breast cancer intellectual disability lung cancer pancreatic ductal adenocarcinoma
B, D, F, H, J B, D, F, H, J, L	Pharos: P05166	propionic acidemia Propionic acidemia, type II attention deficit hyperactivity disorder, inattentive type heart disease inherited organic acidemia lung adenocarcinoma metabolic syndrome X non-small cell lung carcinoma plasma cell myeloma breast cancer intellectual disability lung cancer myocardial infarction ovarian cancer Sjogren syndrome propionic acidemia Propionic acidemia, type II attention deficit hyperactivity disorder, inattentive type heart disease inherited organic acidemia lung adenocarcinoma metabolic syndrome X non-small cell lung carcinoma plasma cell myeloma breast cancer intellectual disability lung cancer myocardial infarction ovarian cancer Sjogren syndrome inborn errors of metabolism lung carcinoma non-alcoholic fatty liver disease pancreatic ductal adenocarcinoma

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.