PANK2 phosphorylates PanK

Stable Identifier
Reaction [transition]
Homo sapiens
Pantothenate is phosphorylated [PANK2]
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Pantothenate kinase 2 catalyzes the reaction of ATP and pantothenate to form ADP and phosphopantothenate. 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). Recent 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.Pantothenate is phosphorylated by pantothenate kinase (PANK). 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
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
Catalyst Activity

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

Orthologous Events
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