Reactome | PANK2 phosphorylates PanK

PANK2 phosphorylates PanK

Stable Identifier

R-HSA-196857

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol, mitochondrial intermembrane space

Synonyms

(R)-pantothenate + ATP => (R)-4'-phosphopantothenate + ADP + H(+)

ReviewStatus

5/5

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Pantothenate kinase 2 (PANK2) catalyzes the reaction of ATP and pantothenate to form ADP and phosphopantothenate. The reaction is inferred to be negatively regulated by CoA, from studies of the mouse enzyme (Zhang et al. 2005). While pantothenate kinase 2 co-purifies with mitocondria, its precise location within the mitochondrion has not been established (Hortnagel et al. 2003; Johnson et al. 2004). Work by Leonardi et al. (2007) supports a model in which the enzyme is located in the intermembrane space, hence freely accessible to small molecules from the cytosol. Deficiencies in PANK2 cause a progressive neurodegenerative disorder associated with iron accumulation in the brain, but the relationship between disease symptoms and pantothenate metabolism remains unclear (Zhou et al. 2001; Zhang et al. 2006).

Literature References

PubMed ID	Title	Journal	Year
16040613	Feedback regulation of murine pantothenate kinase 3 by coenzyme A and coenzyme A thioesters Zhang, YM, Rock, CO, Jackowski, S	J Biol Chem	2005
11479594	A novel pantothenate kinase gene (PANK2) is defective in Hallervorden-Spatz syndrome Hayflick, SJ, Johnson, MA, Zhou, B, Levinson, B, Westaway, SK, Gitschier, J	Nat Genet	2001
12554685	An isoform of hPANK2, deficient in pantothenate kinase-associated neurodegeneration, localizes to mitochondria Meitinger, T, Hortnagel, K, Prokisch, H	Hum Mol Genet	2003
17242360	Activation of human mitochondrial pantothenate kinase 2 by palmitoylcarnitine Leonardi, R, Zhang, YM, Rock, CO, Jackowski, S	Proc Natl Acad Sci U S A	2007
16272150	Biochemical properties of human pantothenate kinase 2 isoforms and mutations linked to pantothenate kinase-associated neurodegeneration Zhang, YM, Rock, CO, Jackowski, S	J Biol Chem	2006
15105273	Mitochondrial localization of human PANK2 and hypotheses of secondary iron accumulation in pantothenate kinase-associated neurodegeneration Hayflick, SJ, Johnson, MA, Ching, KH, Parker, SM, Westaway, SK, Kuo, YM, Gitschier, J	Ann N Y Acad Sci	2004