Protein Domain : IPR006099

Type:  Domain Name:  Methylmalonyl-CoA mutase, alpha/beta chain, catalytic
Description:  Methylmalonyl-CoA mutase () (MCM) [] is an adenosylcobalamin (vitamin B12) dependent enzyme that catalyzes the isomerization between methylmalonyl-CoA and succinyl-CoA. MCM is involved in various catabolic or biosynthetic pathways; for example in man it is involved in the degradation of several amino acids, odd-chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle; while in some bacteria it is involved in the synthesis of propionate from tricarboxylic acid-cycle intermediates.Deficiency of MCM in man causes an often fatal disorder of organic acid metabolism termed methylmalonic acidemia. The sequences of eukaryotic and prokaryotic MCM are rather well conserved. In eukaryotes MCM is located in the mitochondrial matrix and is a homodimer of a polypeptide chain of about 710 amino acids. In bacteria MCM is a dimer of two non-identical, yet structurally related chains. This family also includes an Escherichia coliprotein (gene sbm) whose function is not yet known.A small degree of similarity is said [] to exist between MCM and the large subunit of the adenosylcobalamin-dependent enzyme ethanolamine ammonia-lyase, but this similarity is so weak that these two type of enzymes can not be detected by a single pattern. Short Name:  MeMalonylCoA_mutase_a/b_cat
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1 Child Features

DB identifier Type Name
IPR006098 Domain Methylmalonyl-CoA mutase, alpha chain, catalytic

1 Contains

DB identifier Type Name
IPR014348 Domain Cobalamin (vitamin B12)-dependent enzyme, catalytic subdomain

2 Cross Referencess

Identifier
PF01642
PS00544

1 Found In

DB identifier Type Name
IPR004608 Family Methylmalonyl-CoA mutase, beta chain

3 GO Annotations

GO Term Gene Name
GO:0016866 IPR006099
GO:0031419 IPR006099
GO:0008152 IPR006099

3 Ontology Annotations

GO Term Gene Name
GO:0016866 IPR006099
GO:0031419 IPR006099
GO:0008152 IPR006099

1 Parent Features

DB identifier Type Name
IPR016176 Domain Cobalamin (vitamin B12)-dependent enzyme, catalytic

2 Proteins

DB identifier UniProt Accession Secondary Identifier Organism Name Length
Pum0277s0010.1.p A0A1X6P195 PAC:38013459 Porphyra umbilicalis 707  
Bobra.0226s0026.1.p PAC:40719122 Botryococcus braunii 750  

2 Publications

First Author Title Year Journal Volume Pages PubMed ID
            1975493
            2197274