Pantothenate kinase

from Wikipedia, the free encyclopedia
Pantothenate kinase
Identifier
Gene name (s) PANK1 , PANK2 , PANK3 , PANK4
Enzyme classification
EC, category 2.7.1.33 ,  kinase
Response type Phosphorylation
Substrate Pantothenic acid + ATP
Products Phosphopantothenate + ADP
Occurrence
Parent taxon Creature

Pantothenate kinases are enzymes that phosphorylate pantothenic acid . This is the first reaction step in the biosynthesis of coenzyme A in all living things. Four paralogues of the enzyme are known in humans, each of which has several isoforms (see table). Mutations in the PANK2 gene are responsible for the very rare neurodegeneration with iron deposition in the brain .

Catalyzed equilibrium

Pantothenate+ ATP   + ADP  Phosphopantothenate

(R) -Pantothenate is converted to 4 '- (R) -phosphopantothenate and vice versa.

regulation

The various paralogues are regulated by different mechanisms. While hPanK1 is inhibited by the presence of acetyl-CoA , longer-chain CoA derivatives inhibit the enzyme hPanK3.

Human enzymes

Gene (HGNC) Protein (UniProt) Number Isoforms Cell compartment Tissue type
PANK1 Q8TE04 4th Cytosol Isoform 1: brain, heart, kidneys, liver, skeletal muscles, testes.
PANK2 Q9BZ23 3 Mitochondria Ubiquitous
PANK3 Q9H999 1 Cytosol liver
PANK4 Q9NVE7 1 Cytosol Ubiquitous, especially muscles

Individual evidence

  1. ↑ UniProt Q9BZ23
  2. ↑ Zhang YM, Rock CO, Jackowski S: Feedback regulation of murine pantothenate kinase 3 by coenzyme A and coenzyme A thioesters . In: J. Biol. Chem. . 280, No. 38, September 2005, pp. 32594-601. doi : 10.1074 / jbc.M506275200 . PMID 16040613 .
  3. ↑ Leonardi R, Zhang YM, Lykidis A, Rock CO, Jackowski S: Localization and regulation of mouse pantothenate kinase 2 . In: FEBS Lett . . 581, No. 24, October 2007, pp. 4639-44. doi : 10.1016 / j.febslet.2007.08.056 . PMID 17825826 . PMC 2034339 (free full text).

Web links

Wikibooks: Biochemistry and Pathobiochemistry: Pantothenate Metabolism  - Learning and Teaching Materials