Annotations: 8DYJ

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A [auth B]	PF02310	B12 binding domain (B12-binding)	B12 binding domain	This domain binds to B12 (adenosylcobamide)[1-3], it is found in several enzymes, such as glutamate mutase Swiss:Q05488, methionine synthase Swiss:Q99707 and methylmalonyl-CoA mutase Swiss:P22033. It contains a conserved DxHxxGx(41)SxVx(26)GG motif, ...	This domain binds to B12 (adenosylcobamide)[1-3], it is found in several enzymes, such as glutamate mutase Swiss:Q05488, methionine synthase Swiss:Q99707 and methylmalonyl-CoA mutase Swiss:P22033. It contains a conserved DxHxxGx(41)SxVx(26)GG motif, which is important for B12 binding [2].	Domain
A [auth B]	PF01642	Methylmalonyl-CoA mutase (MM_CoA_mutase)	Methylmalonyl-CoA mutase	-	Family

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A [auth B]	Methylmalonyl-CoA mutase, mitochondrial	methylmalonyl-CoA mutase activity isomerase activity catalytic activity intramolecular transferase activity identical protein binding protein binding binding protein homodimerization activity protein dimerization activity vitamin binding cobalamin binding organic cyclic compound binding tetrapyrrole binding heterocyclic compound binding methylmalonyl-CoA mutase activity isomerase activity catalytic activity intramolecular transferase activity identical protein binding protein binding binding protein homodimerization activity protein dimerization activity vitamin binding cobalamin binding organic cyclic compound binding tetrapyrrole binding heterocyclic compound binding small molecule binding cation binding ion binding metal ion binding pyrophosphatase activity GTPase activity ribonucleoside triphosphate phosphatase activity hydrolase activity, acting on acid anhydrides, in phosphorus-containing anhydrides hydrolase activity hydrolase activity, acting on acid anhydrides modified amino acid binding	homocysteine metabolic process organonitrogen compound metabolic process oxoacid metabolic process sulfur amino acid metabolic process cellular metabolic process carboxylic acid metabolic process amino acid metabolic process primary metabolic process organic acid metabolic process alpha-amino acid metabolic process non-proteinogenic amino acid metabolic process metabolic process small molecule metabolic process cellular process homocysteine metabolic process organonitrogen compound metabolic process oxoacid metabolic process sulfur amino acid metabolic process cellular metabolic process carboxylic acid metabolic process amino acid metabolic process primary metabolic process organic acid metabolic process alpha-amino acid metabolic process non-proteinogenic amino acid metabolic process metabolic process small molecule metabolic process cellular process sulfur compound metabolic process fatty acid metabolic process short-chain fatty acid metabolic process propionate metabolic process, methylmalonyl pathway cellular lipid metabolic process monocarboxylic acid metabolic process propionate metabolic process lipid metabolic process positive regulation of molecular function regulation of molecular function positive regulation of hydrolase activity regulation of catalytic activity regulation of hydrolase activity biological regulation positive regulation of catalytic activity regulation of GTPase activity positive regulation of GTPase activity nucleoside bisphosphate metabolic process ribonucleoside bisphosphate biosynthetic process succinyl-CoA biosynthetic process nucleotide biosynthetic process organonitrogen compound biosynthetic process carbohydrate derivative metabolic process purine ribonucleotide biosynthetic process nucleobase-containing compound metabolic process ribonucleotide biosynthetic process phosphate-containing compound metabolic process thioester metabolic process phosphorus metabolic process ribose phosphate biosynthetic process nucleoside phosphate metabolic process amide biosynthetic process nucleotide metabolic process sulfur compound biosynthetic process biosynthetic process organophosphate biosynthetic process nucleoside phosphate biosynthetic process purine ribonucleotide metabolic process purine nucleotide biosynthetic process ribose phosphate metabolic process purine nucleoside bisphosphate metabolic process ribonucleoside bisphosphate metabolic process thioester biosynthetic process cellular biosynthetic process purine nucleotide metabolic process acyl-CoA metabolic process ribonucleotide metabolic process nucleobase-containing small molecule metabolic process purine-containing compound biosynthetic process acyl-CoA biosynthetic process purine nucleoside bisphosphate biosynthetic process amide metabolic process succinyl-CoA metabolic process carbohydrate derivative biosynthetic process nucleoside bisphosphate biosynthetic process nucleobase-containing compound biosynthetic process purine-containing compound metabolic process organophosphate metabolic process developmental process multicellular organism development multicellular organismal process anatomical structure development post-embryonic development	membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle membrane-enclosed lumen mitochondrial matrix intracellular organelle lumen organelle lumen

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A [auth B]	IPR006098	Methylmalonyl-CoA mutase, alpha chain, catalytic	Domain
A [auth B]	IPR006158	Cobalamin (vitamin B12)-binding domain	Domain
A [auth B]	IPR006099	Methylmalonyl-CoA mutase, alpha/beta chain, catalytic	Domain
A [auth B]	IPR036724	Cobalamin-binding domain superfamily	Homologous Superfamily
A [auth B]	IPR006159	Methylmalonyl-CoA mutase, C-terminal	Domain
A [auth B]	IPR016176	Cobalamin (vitamin B12)-dependent enzyme, catalytic	Homologous Superfamily

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A [auth B]	Pharos: P22033	methylmalonic acidemia methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency inborn errors of metabolism obesity disorder atypical teratoid rhabdoid tumor lung cancer Methylmalonic Aciduria, mut(-) Type osteosarcoma psoriasis vitamin B12-unresponsive methylmalonic acidemia type mut0 cancer inherited organic acidemia ovarian cancer ulcerative colitis glioblastoma methylmalonic acidemia methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency inborn errors of metabolism obesity disorder atypical teratoid rhabdoid tumor lung cancer Methylmalonic Aciduria, mut(-) Type osteosarcoma psoriasis vitamin B12-unresponsive methylmalonic acidemia type mut0 cancer inherited organic acidemia ovarian cancer ulcerative colitis glioblastoma large cell medulloblastoma pancreatic ductal adenocarcinoma vitamin B12-unresponsive methylmalonic acidemia type mut-

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.