isovaleryl-CoA dehydrogenase

イソバレリルCoA脱水素酵素、イソバレリルCoAデヒドロゲナーゼ

WordNet

1. the 3rd letter of the Roman alphabet (同)c
2. (music) the keynote of the scale of C major
3. a general-purpose programing language closely associated with the UNIX operating system

PrepTutorEJDIC

1. carbonの化学記号
2. cobaltの化学記号

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/12/29 23:45:41」(JST)

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[Wiki en表示]

isovaleryl-CoA dehydrogenase
Isovaleryl-CoA dehydrogenase tetramer, Human
Identifiers
EC number	1.3.8.4
CAS number	37274-61-6
Databases
IntEnz	IntEnz view
BRENDA	BRENDA entry
ExPASy	NiceZyme view
KEGG	KEGG entry
MetaCyc	metabolic pathway
PRIAM	profile
PDB structures	RCSB PDB PDBe PDBsum
Gene Ontology	AmiGO / EGO
Search PMC articles PubMed articles NCBI proteins

In enzymology, an isovaleryl-CoA dehydrogenase (EC 1.3.8.4) 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 has been solved for this class of enzymes, with the PDB accession code 1IVH.

References

• BACHHAWAT BK, ROBINSON WG, COON MJ (1956). "Enzymatic carboxylation of beta-hydroxyisovaleryl coenzyme A". J. Biol. Chem. 219 (2): 539–50. PMID 13319276. 
• Ikeda Y, Tanaka K (1983). "Purification and characterization of isovaleryl coenzyme A dehydrogenase from rat liver mitochondria". J. Biol. Chem. 258 (2): 1077–85. PMID 6401713. 
• Tanaka K, Budd MA, Efron ML, Isselbacher KJ (1966). "Isovaleric acidemia: a new genetic defect of leucine metabolism". Proc. Natl. Acad. Sci. U.S.A. 56 (1): 236–42. doi:10.1073/pnas.56.1.236. PMC 285701. PMID 5229850. 

UpToDate Contents

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English Journal

• Genotype-Phenotype Correlation in Patients with Isovaleric Acidemia: Comparative Structural Modelling and Computational Analysis of Novel Variants.

• Zaki OK1, Priya Doss C G2, Ali SA3, Murad GG3, Elashi SA3, Ebnou MS3, Thirumal Kumar D2, Khalifa O1, Gamal R1, El Abd HSA1, Nasr BN3, Zayed H3.
• Human molecular genetics.Hum Mol Genet.2017 May 23. doi: 10.1093/hmg/ddx195. [Epub ahead of print]
• PMID 28535199

• Genotype and phenotype characterization in a Spanish cohort with isovaleric acidemia.

• Couce ML1, Aldamiz-Echevarría L2, Bueno MA3, Barros P4, Belanger-Quintana A5, Blasco J6, García-Silva MT7, Márquez-Armenteros AM8, Vitoria I9, Vives I10, Navarrete R11, Fernández-Marmiesse A1, Pérez B11, Pérez-Cerdá C11.
• Journal of human genetics.J Hum Genet.2017 Mar;62(3):355-360. doi: 10.1038/jhg.2016.144. Epub 2016 Dec 1.
• PMID 27904153

• [Pancytopenia and metabolic decompensation in a neonate].

• Tan JQ1, Chen DY, Mo ZQ, Li ZT, Huang JW, Cai R, Yan TZ.
• Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics.Zhongguo Dang Dai Er Ke Za Zhi.2016 Nov;18(11):1150-1153.
• PMID 27817783

Japanese Journal

• Phenotypic Variability and Newly Identified Mutations of the IVD Gene in Japanese Patients with Isovaleric Acidemia

• The Tohoku Journal of Experimental Medicine 236(2), 103-106, 2015
• NAID 130005072136

• 好塩性アーキアの膜脂質イソプレノイド生成機構-ロイシン-メバロン酸経路と関連酵素の存在

• 日本地球化学会年会要旨集 60(0), 139, 2013
• NAID 130004593890

• The Pathway of Leucine to Mevalonate in Halophilic Archaea : Efficient Incorporation of Leucine into Isoprenoidal Lipid with the Involvement of Isovaleryl-CoA Dehydrogenase in Halobacterium salinarum

• Bioscience, biotechnology, and biochemistry 74(2), 443-446, 2010-02-23
• NAID 10027552008

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★リンクテーブル★

リンク元	「イソバレリルCoA脱水素酵素」「イソバレリルCoAデヒドロゲナーゼ」
関連記事	「C」「CoA」「dehydrogenase」「dehydrogenases」「COA」

「イソバレリルCoA脱水素酵素」

　　[★]

英

isovaleryl-CoA dehydrogenase

関

イソバレリルCoAデヒドロゲナーゼ

「イソバレリルCoAデヒドロゲナーゼ」

　　[★]

英

isovaleryl-CoA dehydrogenase

関

イソバレリルCoA脱水素酵素

「C」

　　[★]

• 炭素 carbon
• シトシン cytosine
• システイン cysteine
• シチジン
• セルシウス度 摂氏 degrees Celsius degree C
• 産婦人科領域で「子宮外頚 子宮外頚部 exocervix」を表現するための略語

　 　 　 　

「CoA」

　　[★]

• 大動脈縮窄症 coactation of the aorta
• 補酵素A coenzyme A

「dehydrogenase」

　　[★] 脱水素酵素 デヒドロゲナーゼ

「dehydrogenases」

　　[★] 脱水素酵素 デヒドロゲナーゼ

「COA」

　　[★] 補酵素A coenzyme A CoA