| Gene Symbol |
MCCC1
|
| Entrez Gene |
56922
|
| Alt Symbol |
MCC-B, MCCA
|
| Species |
Human
|
| Gene Type |
protein-coding
|
| Description |
methylcrotonoyl-CoA carboxylase 1 (alpha)
|
| Other Description |
3-methylcrotonyl-CoA carboxylase 1|3-methylcrotonyl-CoA carboxylase biotin-containing subunit|3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha|MCCase subunit alpha|methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial|methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)
|
| Swissprots |
Q59ES4 Q9NS97 Q96RQ3 Q9H959
|
| Accessions |
EAW78339 EAW78340 Q96RQ3 AB029826 BAA99407 AB209737 BAD92974 AF297332 AAK67986 AF310339 AAG50245 AF310972 AAG53095 AK023051 BAB14377 AK098411 AK294882 BAG57977 AK295293 BAG58277 AK316428 BAH14799 AL442091 AL532496 AL556872 BC004187 AAH04187 BC004214 AAH04214 BC036395 BC042453 BF795651 CR749608 CAH18403 DA487016 XM_006713702 XP_006713765 XM_011512992 XP_011511294 XM_011512993 XP_011511295 XR_241502 XR_924159 NM_001293273 NP_001280202 NM_020166 NP_064551 NR_120639 NR_120640
|
| Function |
Biotin-attachment 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. {ECO:0000269|PubMed:17360195}.
|
| Subcellular Location |
Mitochondrion matrix {ECO:0000269|PubMed:16023992}.
|
| Top Pathways |
Valine, leucine and isoleucine degradation
|
