Isobutyryl-CoA

	Isobutyryl-CoA Isobutyryl-coenzyme A is a starting material for many natural products derived from Poly-Ketide Synthase (PKS) assembly lines, as well as PKS-NRPS hybrid assembly lines. These products can often be used as antibiotics. Notably, it is also an intermediate in the metabolism of the amino acid Valine, and structurally similar to intermediates in the catabolism of other small amino acids. See also * Isobutyryl-CoA mutase * Isobutyryl-coenzyme A dehydrogenase deficiency Coenzymes {{organic-compound-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu]
	Isobutyryl-CoA Mutase In enzymology, an isobutyryl-CoA mutase () is an enzyme that catalyzes the chemical reaction :2-methylpropanoyl-CoA \rightleftharpoons butanoyl-CoA Hence, this enzyme has one substrate, 2-methylpropanoyl-CoA, and one product, butanoyl-CoA. This enzyme belongs to the family of isomerases, specifically those intramolecular transferases transferring other groups. The systematic name of this enzyme class is 2-methylpropanoyl-CoA CoA-carbonylmutase. Other names in common use include isobutyryl coenzyme A mutase, and butyryl-CoA:isobutyryl-CoA mutase. It uses adenosylcobalamin as a cofactor, which is bound at the enzyme's vitamin B12-binding domain. The mechanism of action of the enzyme is to generate a 5′-deoxyadenosyl radical A deoxyadenosyl radical is a free radical that is structurally related to adenosine by removal of a 5′-hydroxy group from adenosine. This radical occurs in nature as a reactive intermediate. It is generated by radical SAM Radical SAM is a desig ... b ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu]
	Valine Valine (symbol Val or V) is an α-amino acid that is used in the biosynthesis of proteins. 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 isopropyl 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, beans and legumes. It is encoded by all codons starting with GU (GUU, GUC, GUA, and GUG). History and etymology Valine was first isolated from casein in 1901 by Hermann Emil Fischer. The name valine comes from valeric acid, which in turn is named after the plant valerian due to the presence of the acid in the roots of the plant. Nomenclature According to IUPAC, carbon atoms forming valine are numbered sequentially s ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu]
	Isobutyryl-coenzyme A Dehydrogenase Deficiency Isobutyryl-coenzyme A dehydrogenase deficiency is a rare metabolic disorder in which the body is unable to process certain amino acids properly. People with this disorder have inadequate levels of an enzyme that helps break down the amino acid valine, resulting in a buildup of valine in the urine, a symptom called valinuria. Genetics Defects in the ''ACAD8'' gene cause isobutyryl-coenzyme A dehydrogenase deficiency. The ''ACAD8'' gene provides instructions for making an enzyme that plays an essential role in breaking down proteins from the diet. Specifically, the enzyme is responsible for processing valine, an amino acid that is part of many proteins. If a mutation in the ACAD8 gene reduces or eliminates the activity of this enzyme, the body is unable to break down valine properly. As a result, poor growth and reduced energy production may occur. This disorder is inherited in an autosomal recessive pattern, which means the defective gene is located on an autosome, and two c ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu]