Protein Name: Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
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UniprotKB/SwissProt ID: MCCB_HUMAN (Q9HCC0)
Gene Name: MCCC2
Synonyms: MCCB
Organism: Homo sapiens (Human).
Function: Carboxyltransferase subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3- methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism.
Other Modifications: View all modification sites in dbPTM
Protein Subcellular Localization: Mitochondrion matrix.
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Protein disease:
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| Disease database |
Database Entry |
Disease information | | HPRD | 01952 | 3-@methylcrotonyl-CoA carboxylase 2 deficiency |
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Network with metabolic pathway:
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Graphical Visualization of S-nitrosylation Sites:
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| Overview of Protein S-nitrosylation Sites with Functional and Structural Information |  |
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