β-methylcrotonyl-CoA

	β-methylcrotonyl-CoA 3-Methylcrotonyl-CoA or β-Methylcrotonyl-CoA is an intermediate in the metabolism of leucine. It is found in mitochondria, where it is formed from isovaleryl-coenzyme A by isovaleryl coenzyme A dehydrogenase. It then reacts with CO2 to yield 3-Methylcrotonyl-CoA carboxylase. 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 ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu]
	Metabolic Intermediate Metabolic intermediates are molecules that are the precursors or metabolites of biologically significant molecules. Although these intermediates are of relatively minor direct importance to cellular function, they can play important roles in the allosteric regulation of enzymes. Clinical significance Some can be useful in measuring rates of metabolic processes (for example, 3,4-dihydroxyphenylacetic acid or 3-aminoisobutyrate). Because they can represent unnatural points of entry into natural metabolic pathways, some (such as AICA ribonucleotide) are of interest to researchers in developing new therapies. See also * Metabolism Metabolism {{chem-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu]
picture info	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- carboxylic acid group (which is in the deprotonated −COO− form under biological conditions), and a side chain isobutyl group, making it a non-polar aliphatic amino acid. It is essential in humans, meaning the body cannot synthesize it: it must be obtained from the diet. Human dietary sources are foods that contain protein, such as meats, dairy products, soy products, and beans and other legumes. It is encoded by the codons UUA, UUG, CUU, CUC, CUA, and CUG. Like valine and isoleucine, leucine is a branched-chain amino acid. The primary metabolic end products of leucine metabolism are acetyl-CoA and acetoacetate; consequently, it is one of the two exclusively ketogenic amino acids, with lysine being the other. It is the most import ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu]
	Isovaleryl-coenzyme A Isovaleryl-coenzyme A, also known as isovaleryl-CoA, is an intermediate in the metabolism of branched-chain amino acids. Leucine metabolism See also * 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 ... References Thioesters of coenzyme A {{biochem-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu]
	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-methylbutanoyl-CoA and acceptor, whereas its two products are 3-methylbut-2-enoyl-CoA and reduced acceptor. This enzyme belongs to the family of oxidoreductases, specifically those acting on the CH-CH group of donor with other acceptors. The systematic name of this enzyme class is 3-methylbutanoyl-CoA:acceptor oxidoreductase. Other names in common use include isovaleryl-coenzyme A dehydrogenase, isovaleroyl-coenzyme A dehydrogenase, and 3-methylbutanoyl-CoA:(acceptor) oxidoreductase. This enzyme participates in valine, leucine and isoleucine degradation. It employs one cofactor, FAD. Structural studies As of late 2007, only one structure A structure is an arrangement and organization of interrelated elements in a material object or sys ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu]
	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 of a carbon adjacent to a carbonyl group performing the fourth step in processing leucine, an essential amino acid. Structure Gene Human MCC is a biotin dependent mitochondrial enzyme formed by the two subunits MCCCα and MCCCβ, encoded by ''MCCC1'' and ''MCCC2'' respectively. MCCC1 gene has 21 exons and resides on chromosome 3 at q27. MCCC2 gene has 19 exons and resides on chromosome 5 at q12-q13. Protein The enzyme contains α and β subunits. Human MCCCα is composed of 725 amino acids which harbor a covalently bound biotin essential for the ATP-dependent carboxylation; MCCCβ has 563 amino acids that possess carboxyltransferase activity which presumably is essential for binding to 3-methylcrotonyl CoA. The MCC holoenzyme is thou ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu]