|Other Names||Pantothenate kinase 2, mitochondrial, hPanK2, Pantothenic acid kinase 2, PANK2, C20orf48|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP7160a was selected from the N-term region of human PANK2. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.|
|Format||The synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml deionized water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||May be the master regulator of the CoA biosynthesis.|
|Cellular Location||Isoform 1: Mitochondrion. Isoform 3: Cytoplasm.|
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Provided below are standard protocols that you may find useful for product applications.
Pantothenate kinase is an essential regulatory enzyme in CoA biosynthesis, catalyzing the cytosolic phosphorylation of pantothenate (vitamin B5), N-pantothenoylcysteine, and pantetheine. CoA is the major acyl carrier, playing a central role in intermediary and fatty acid metabolism. In both yeast and fly, each with only 1 pantothenate kinase gene, the null mutant is inviable. Mutations in PANK2 are the cause of pantothenate kinase-associated neurodegeneration (PKAN), formerly known as Hallervorden-Spatz syndrome (HSS). PKAN is an autosomal recessive neurodegenerative disorder associated with iron accumulation in the brain. Mutations in PANK2 are the cause of hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP).
Neurology 58: 1673-1674, 2002. Hum. Molec. Genet. 12: 321-327, 2003. Neurology 61: 1423-1426, 2003. Neurology 64: 1810-1812, 2005. Nature Genet. 28: 345-349, 2001.
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