|Application ||WB, E|
|Calculated MW||62681 Da|
|Other Names||Pantothenate kinase 2, mitochondrial, hPanK2, Pantothenic acid kinase 2, PANK2, C20orf48|
|Target/Specificity||This PANK2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 65-95 amino acids from the N-terminal region of human PANK2.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||PANK2 Antibody (N-term) is for research use only and 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.|
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.