C20orf48; Pantothenate kinase 2, mitochondrial; hPanK2; Pantothenic acid kinase 2

For Research Use Only. Not for use in diagnostic procedures.

Synthetic

>85%

Lyophilized powder

Shipped at 4 degree C. Store at -20 degree C for one year.

Small volumes of PANK2 blocking peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

The peptide is used to block Anti-PANK2 Antibody reactivity.

Blocking (BL)

Blocking Peptide to the diluted primary antibody in a molar ratio of 10:1 (peptide to antibody) and incubate the mixture at 4 degree C for overnight or at room temperature for 2 hours.

50,582 Da

pantothenate kinase 2

pantothenate kinase 2, mitochondrial; pantothenic acid kinase 2; Hallervorden-Spatz syndrome

Pantothenate kinase 2, mitochondrial

C20orf48; hPanK2??[Similar Products]

PANK2_HUMAN

This gene encodes a protein belonging to the pantothenate kinase family and is the only member of that family to be expressed in mitochondria. Pantothenate kinase is a key regulatory enzyme in the biosynthesis of coenzyme A (CoA) in bacteria and mammalian cells. It catalyzes the first committed step in the universal biosynthetic pathway leading to CoA and is itself subject to regulation through feedback inhibition by acyl CoA species. Mutations in this gene are associated with HARP syndrome and pantothenate kinase-associated neurodegeneration (PKAN), formerly Hallervorden-Spatz syndrome. Alternative splicing, involving the use of alternate first exons, results in multiple transcripts encoding different isoforms. [provided by RefSeq, Jul 2008]

PANK2: a pantothenate kinase that catalyzes the first committed step in the biosynthesis of coenzyme A, an essential cofactor in cellular metabolism. PANK2 is predominantly localized in mitochondria in primates. Defects in PANK2 are the cause of pantothenate kinase-associated neurodegeneration (PKAN), an autosomal recessive neurodegenerative disorder associated with iron accumulation in the brain. Three isoforms of the human PANK2, produced by alternative splicing, have been reported.

Protein type: EC 2.7.1.33; Cofactor and Vitamin Metabolism - pantothenate and CoA biosynthesis; Mitochondrial; Kinase, other

Chromosomal Location of Human Ortholog: 20p13

Cellular Component: mitochondrial intermembrane space; cytosol

Molecular Function: pantothenate kinase activity; ATP binding

Biological Process: coenzyme biosynthetic process; coenzyme A biosynthetic process; vitamin metabolic process; regulation of mitochondrial membrane potential; pantothenate metabolic process; aerobic respiration; phosphorylation; water-soluble vitamin metabolic process; spermatid development

Disease: Hypoprebetalipoproteinemia, Acanthocytosis, Retinitis Pigmentosa, And Pallidal Degeneration; Neurodegeneration With Brain Iron Accumulation 1

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