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).
Hayflick, SJ, Johnson, MA, Zhou, B, Levinson, B, Westaway, SK, Gitschier, J
Meitinger, T, Hortnagel, K, Prokisch, H
Leonardi, R, Zhang, YM, Rock, CO, Jackowski, S
Zhang, YM, Rock, CO, Jackowski, S
Hayflick, SJ, Johnson, MA, Ching, KH, Parker, SM, Westaway, SK, Kuo, YM, Gitschier, J
pantothenate kinase activity of PANK2(111-570) [mitochondrial intermembrane space]
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