Branched-chain alpha-keto acid dehydrogenase complex

Branched-chain alpha-keto acid dehydrogenase complex

protein
Name=2-oxoisovalerate dehydrogenase (lipoamide)
caption=


width=
HGNCid=8523
Symbol=OVD1A
AltSymbols=
EntrezGene=5014
OMIM=
RefSeq=
UniProt=
PDB=
ECnumber=1.2.4.4
Chromosome=
Arm=
Band=
LocusSupplementaryData=
The branched-chain α-keto acid dehydrogenase complex is a combination of enzymes responsible for the degradation of the branched chain amino acids. Examples of these include valine, isoleucine, and leucine.

Cofactors

This complex requires the following 5 cofactors:
*Thiamine diphosphate
*FAD
*NAD+
*Lipoate
*Coenzyme A

imilar enzymes

This complex is also analogous to the alpha-ketoglutarate dehydrogenase complex in the citric acid cycle and the pyruvate dehydrogenase complex creating acetyl-CoA prior to the citric acid cycle.

Pathology

A deficiency in any of the enzymes of this complex as well as an inhibition of the complex as a whole is responsible for maple syrup urine disease.

This enzyme is an autoantigen recognized in primary biliary cirrhosis, a form of acute liver failure. These antibodies appear to recognize oxidized protein that has resulted from inflamatory immune responses. Some of these inflamatory responses are explained by gluten sensitivity.cite journal | author = Leung PS, Rossaro L, Davis PA, "et al" | title = Antimitochondrial antibodies in acute liver failure: Implications for primary biliary cirrhosis | journal = | volume = | issue = | pages = | year = 2007 | pmid = 17657817 | doi = 10.1002/hep.21828] . Other mitochondrial autoantigensinclude pyruvate dehydrogenase and branched chain oxoglutarate dehydrogenase, which are antigens recognized by anti-mitochondrial antibodies.

References

External links

*
*


Wikimedia Foundation. 2010.

Игры ⚽ Поможем написать курсовую

Look at other dictionaries:

  • Branched-chain amino acids — The phrase branched chain amino acids or BCAA is sometimes used to refer to the amino acids having aliphatic side chains that are non linear.These are leucine, isoleucine and valine. The combination of these three essential amino acids make up… …   Wikipedia

  • Oxoglutarate dehydrogenase complex — The oxoglutarate dehydrogenase complex (OGDC) or α ketoglutarate dehydrogenase complex is an enzyme complex, most commonly known for its role in the citric acid cycle. Contents 1 Units 2 Properties 2.1 Metabolic pathways 2.2 …   Wikipedia

  • Pyruvate dehydrogenase complex — (PDC) is a complex of three enzymes that transform pyruvate into acetyl CoA by a process called pyruvate decarboxylation. Acetyl CoA may then be used in the citric acid cycle to carry out cellular respiration, and this complex links the… …   Wikipedia

  • Amino acid — This article is about the class of chemicals. For the structures and properties of the standard proteinogenic amino acids, see Proteinogenic amino acid. The generic structure of an alpha amino acid in its unionized form …   Wikipedia

  • Pyruvate dehydrogenase — Crystallographic structure of pyruvate dehydrogenase (PDH). PH is a six domain dimer with α (blue), α’ (yellow), β (red), and β’ (teal) regions denoted by the different colors. Thiamine pyrophosphate (TPP) is shown in grey ball and stick form,… …   Wikipedia

  • Dihydrolipoamide dehydrogenase — PDB rendering based on 1zy8 …   Wikipedia

  • Oxoglutarate dehydrogenase — Protbox Name=2 oxoglutarate dehydrogenase E1 component (α Ketoglutarate dehydrogenase) Photo= Caption= HGNCid = 8124 Symbol = OGDH AltSymbols = Names= Alpha ketoglutarate dehydrogenase Chromosome = 7 Arm = p Band = 13 LocusSupplementaryData = p11 …   Wikipedia

  • Cholesterol side-chain cleavage enzyme — Cytochrome P450, family 11, subfamily A, polypeptide 1 Identifiers Symbols CYP11A1; CYP11A; P450SCC External IDs …   Wikipedia

  • Cytochrome b6f complex — Crystal structure of the cytochrome b6f complex from C. reinhardtii (1q90). Hydrocarbon boundaries of the lipid bilayer are shown by red and blue dots …   Wikipedia

  • Aldehyde dehydrogenase — Not to be confused with aldehyde oxidase. Aldehyde dehydrogenase (NAD+) Monomer of human aldehyde dehydrogenase 2 (ALDH2) with a space filling model of NAD+ in the active site …   Wikipedia

Share the article and excerpts

Direct link
Do a right-click on the link above
and select “Copy Link”