Anti-PANK2 monoclonal antibody (DCABH-914)

Mouse anti-Human PANK2 monoclonal antibody for WB, IHC-P, FC, ICC/IF Datasheet

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Specifications


Host Species
Mouse
Antibody Isotype
IgG2a
Clone
4I0
Species Reactivity
Rat, Dog, Human, Monkey
Immunogen
Recombinant full length Human PANK2 produced in HEK293T cells (NP_705902).
Conjugate
Unconjugated

Applications


Application Notes
WB: 1/1000 - 1/2000; IHC-P: 1/50; Flow Cyt: 1/100; ICC/IF: 1/100;
*Suggested working dilutions are given as a guide only. It is recommended that the user titrates the product for use in their own experiment using appropriate negative and positive controls.

Target


Alternative Names
PANK2; PANK2 protein; HSS; HARP; PKAN; NBIA1
Entrez Gene ID

Product Background


Gene summary
PANK2 (Pantothenate Kinase 2) is a Protein Coding gene. Diseases associated with PANK2 include neurodegeneration with brain iron accumulation 1 and harp syndrome. Among its related pathways are Metabolism and Metabolism of water-soluble vitamins and cofactors. GO annotations related to this gene include pantothenate kinase activity. An important paralog of this gene is PANK1. 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.
Antigen Description
May be the master regulator of the CoA biosynthesis. Hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP) [MIM:607236]: Rare syndrome with many clinical similarities to PKAN. Note=The disease is caused by mutations affecting the gene represented in this entry. Neurodegeneration with brain iron accumulation 1 (NBIA1) [MIM:234200]: Autosomal recessive neurodegenerative disorder associated with iron accumulation in the brain, primarily in the basal ganglia. Clinical manifestations include progressive muscle spasticity, hyperreflexia, muscle rigidity, dystonia, dysarthria, and intellectual deterioration which progresses to severe dementia over several years. It is clinically classified into classic, atypical, and intermediate phenotypes. Classic forms present with onset in first decade, rapid progression, loss of independent ambulation within 15 years. Atypical forms have onset in second decade, slow progression, maintenance of independent ambulation up to 40 years later. Intermediate forms manifest onset in first decade with slow progression or onset in second decade with rapid progression. Patients with early onset tend to also develop pigmentary retinopathy, whereas those with later onset tend to also have speech disorders and psychiatric features. All patients have the eye of the tiger sign on brain MRI. Pantothenate kinase 2, mitochondrial is an enzyme that in humans is encoded by the PANK2 gene.

Citations


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References


Hartig, MB; Iuso, A; et al. Absence of an Orphan Mitochondrial Protein, C19orf12, Causes a Distinct Clinical Subtype of Neurodegeneration with Brain Iron Accumulation. AMERICAN JOURNAL OF HUMAN GENETICS 89:543-550(2011).

Custom Antibody Labeling


We offer labeled antibodies using our catalogue antibody products and a broad range of intensely fluorescent dyes and labels including HRP, biotin, ALP, Alexa Fluor® dyes, DyLight® Fluor dyes, R-phycoerythrin (R-PE), at scales from less than 100 μg up to 1 g of IgG antibody. Learn More

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