EC Number   |
General Information |
Reference |
|---|
    6.4.1.4 | malfunction |
deficiency of 3-methylcrotonyl-CoA carboxylase causes methylcrotonylglycinuria |
716236 |
| 6.4.1.4 | malfunction |
marginal biotin deficiency reduces the enzymatic activity of MCC, thereby causing impairment in leucine catabolism |
713750 |
| 6.4.1.4 | malfunction |
T-DNA and transposon-tagged mutant alleles of the MCCA and MCCB genes of Arabidopsis thaliana are recovered. Mutations in either MCCA or MCCB block mitochondrial Leu catabolism. Under light deprivation conditions, the hyper-accumulation of Leu, 3-methylcrotonyl CoA and isovaleryl CoA indicates that mitochondrial and peroxisomal Leu catabolism pathways are independently regulated. Block in mitochondrial Leu catabolism is associated with an impaired reproductive growth phenotype. The decreased seed germination phenotype is only observed for homozygous mutant seeds |
728482 |
| 6.4.1.4 | physiological function |
inhibition of MCC2 expression by RNA interference disturbs the flux of carbon (mainly in the form of leucine) toward branched-chain amino acid degradation, resulting in decreased triacylglycerol accumulation. MCC2 inhibition also gives rise to incomplete utilization of nitrogen, thus lowering biomass during the stationary growth phase |
746027 |
