Pantothenate Kinase 2, Mitochondrial, Polyclonal Antibody

Anti -Pantothenate Kinase 2, Mitochondrial (Pantothenic Acid Kinase 2, PANK2, hPanK2, C20orf48, FLJ17232, HSS, HARP, MGC15053, NBIA1, PKAN)

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

Chromosome: 20; NC_000020.10 (3869486..3904502). Location: 20p13

Polyclonal

IgG

Rabbit

Recognizes human PANK2.

Affinity Purified
Purified by Protein G affinity chromatography.

Supplied as a liquid in PBS, 0.09% sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Small volumes of anti-PANK2 antibody 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.

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).

Antibodies; Abs to Protein Kinases

ELISA (EL/EIA), Western Blot (WB)

Suitable for use in ELISA and Western Blot.
Dilution: ELISA: 1:1,000
Western Blot: 1:100-1:500

62,681 Da[Similar Products]

pantothenate kinase 2

pantothenate kinase 2, mitochondrial; hPanK2; OTTHUMP00000030143; OTTHUMP00000030148; pantothenic acid kinase 2; Hallervorden-Spatz syndrome

Pantothenate kinase 2, mitochondrial

C20orf48  [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]

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: Cofactor and Vitamin Metabolism - pantothenate and CoA biosynthesis; EC 2.7.1.33; 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; spermatid development; water-soluble vitamin metabolic process

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

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.