|Western Blot (WB)||1:1000|
|Tested Species reactivity||Human, Mouse|
|Host / Isotype||Rabbit / IgG|
|Immunogen||KLH conjugated synthetic peptide between 65-95 amino acids from the N-terminal region of human PANK2|
|Purification||Size-exclusion - Dialysis, Ammonium sulfate precipitation|
|Contains||0.09% sodium azide|
|Storage conditions||-20° C, Avoid Freeze/Thaw Cycles|
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).
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: Hallervorden-Spatz syndrome; hPANK2; pantothenate kinase 2 (Hallervorden-Spatz syndrome); Pantothenate kinase 2, mitochondrial; Pantothenic acid kinase 2
Gene Aliases: 4933409I19Rik; AI642621; C20orf48; HARP; HSS; NBIA1; PANK2; PKAN
UniProt ID: (Human) Q9BZ23
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