Pantothenate kinase 2; mitochondrial; hPanK2; Pantothenic acid kinase 2; PANK2; C20orf48

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

The synthetic peptide sequence used to generate the antibody 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.

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.

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

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.

May be the master regulator of the CoA biosynthesis.

50,582 Da

pantothenate kinase 2

pantothenate kinase 2, mitochondrial

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

Chromosomal Location of Human Ortholog: 20p13

Cellular Component: cytoplasm; mitochondrial intermembrane space

Molecular Function: pantothenate kinase activity

Biological Process: coenzyme biosynthetic process; regulation of fatty acid metabolic process

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

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