PANK2 phosphorylates PanK

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).

泛酸激酶2（PANK2）催化ATP与泛酸反应生成ADP和磷泛酸。该反应据推测受到辅酶A的负向调控，这一推断基于对小鼠酶的研究（张等，2005年）。尽管泛酸激酶2与线粒体共纯化，但其在线粒体内的精确位置尚未得到确定（霍特纳格尔等，2003年；约翰逊等，2004年）。莱昂纳迪等（2007年）的研究支持了一种模型，即该酶位于线粒体间隙，因此可以自由地被细胞质中的小分子所接触。PANK2的缺乏导致一种与大脑中铁积累相关的进行性神经退行性疾病，但疾病症状与泛酸代谢之间的关系尚不明确（周等，2001年；张等，2006年）。