β-methylcrotonyl-CoA
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3-Methylcrotonyl-CoA or β-Methylcrotonyl-CoA is an intermediate in the metabolism of
leucine Leucine (symbol Leu or L) is an essential amino acid that is used in the biosynthesis of proteins. Leucine is an α-amino acid, meaning it contains an α-amino group (which is in the protonated −NH3+ form under biological conditions), an α- ca ...
. It is found in mitochondria, where it is formed from isovaleryl-coenzyme A by
isovaleryl coenzyme A dehydrogenase In enzymology, an isovaleryl-CoA dehydrogenase () is an enzyme that catalyzes the chemical reaction :3-methylbutanoyl-CoA + acceptor \rightleftharpoons 3-methylbut-2-enoyl-CoA + reduced acceptor Thus, the two substrates of this enzyme are 3-met ...
. It then reacts with CO2 to yield
3-Methylcrotonyl-CoA carboxylase Methylcrotonyl CoA carboxylase (, MCC) (3-methylcrotonyl CoA carboxylase, methylcrotonoyl-CoA carboxylase) is a biotin-requiring enzyme located in the mitochondria. MCC uses bicarbonate as a carboxyl group source to catalyze the carboxylation ...
.


Leucine metabolism


See also

*
Methylcrotonyl-CoA carboxylase Methylcrotonyl CoA carboxylase (, MCC) (3-methylcrotonyl CoA carboxylase, methylcrotonoyl-CoA carboxylase) is a biotin-requiring enzyme located in the mitochondria. MCC uses bicarbonate as a carboxyl group source to catalyze the carboxylation o ...


References

Thioesters of coenzyme A {{biochem-stub