Rabbit PANK2 Polyclonal Antibody | anti-PANK2 antibody

PANK2 Polyclonal Antibody

Cat. Num. AAA3006759

• Gene Names: PANK2; HSS; HARP; PKAN; NBIA1; C20orf48
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: PANK2; Polyclonal Antibody; PANK2 Polyclonal Antibody; Pantothenate kinase 2; mitochondrial; hPanK2; Pantothenic acid kinase 2; C20orf48; anti-PANK2 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: PANK2 polyclonal antibody detects endogenous levels of PANK2 protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 570

• Applicable Applications for anti-PANK2 antibody: Western Blot (WB)
• Application Notes: WB: 1:500-1:1000
• Immunogen: A synthetic peptide corresponding to residues in Human PANK2.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Western Blot (WB)

(Western Blot (WB) analysis of PANK2 polyclonal antibody at 1:500 dilutionLane1:MCF-7 cell lysateLane2:sp2/0 cell lysateLane3:PC12 cell lysate)

Related Product Information for anti-PANK2 antibody

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.

NCBI and Uniprot Product Information

• NCBI GI #: 118572682
• NCBI GeneID: 80025
• UniProt Accession #: Q9BZ23
• Molecular Weight: ~ 50kDa
• NCBI Official Full Name: Pantothenate kinase 2, mitochondrial
• NCBI Official Synonym Full Names: pantothenate kinase 2
• NCBI Official Symbol: PANK2
• NCBI Official Synonym Symbols: HSS; HARP; PKAN; NBIA1; C20orf48
• NCBI Protein Information: pantothenate kinase 2, mitochondrial
• UniProt Protein Name: Pantothenate kinase 2, mitochondrial
• Protein Family: Pantothenate kinase
• UniProt Gene Name: PANK2
• UniProt Synonym Gene Names: C20orf48
• UniProt Entry Name: PANK2_HUMAN

NCBI Description

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]

Uniprot Description

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

Chromosomal Location of Human Ortholog: 20p13

Cellular Component: mitochondrial intermembrane space; cytosol

Molecular Function: pantothenate kinase activity; ATP binding

Biological Process: coenzyme A biosynthetic process; coenzyme 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