PANK2 (pantothenate kinase 2), also known as HSS, HARP, PKAN or NBIA1, is a ubiquitously expressed 570 amino acid member of the pantothenate kinase family of enzymes that are involved in the synthesis of coenzyme A (CoA).
Localized to the cytoplasm and mitochondria, PANK2 is thought to be the chief regulator of CoA biosynthesis, catalyzing the first of five steps in the biosynthetic pathway.
Regulated by feedback inhibition from synthesized CoA, PANK2 catalyzes the ATP-dependent conversion of pantothenate to 4’-phosphopantothenate, thus initiating the first committed step in CoA biosynthesis.
Defects in the gene encoding PANK2 are the cause of pantothenate kinase-associated neurodegeneration (PKAN) and hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa and pallidal degeneration (HARP).
PKAN and HARP are rare disorders characterized by extrapyramidal dysfunction and progressive dementia, both of which are caused by an accumulation of iron in the brain.
PANK2 is expressed as three isoforms due to alternative splicing events.
Applications:Suitable for use in Western Blot.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:1000Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid PBS, 0.1% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of PANK2. Species Crossreactivity: Human, mouse, rat
Isotype：IgG
Calc Applications Abbrev：WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：A synthetic peptide corresponding to residues in Human PANK2.
