PANK2 phosphorylates PanK

Stable Identifier
R-HSA-196857
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
(R)-pantothenate + ATP => (R)-4'-phosphopantothenate + ADP + H(+)
ReviewStatus
5/5
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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
Participants
Participates
Catalyst Activity

pantothenate kinase activity of PANK2(111-570) [mitochondrial intermembrane space]

This event is regulated
Negatively by
Regulator