Overview

  • Product name

  • Description

    Rabbit polyclonal to PANK2
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, IHC-P, ICC/IFmore details
  • Species reactivity

    Reacts with: Mouse, Human
  • Immunogen

    Recombinant fragment corresponding to Human PANK2 aa 437-490.
    Sequence:

    MASRGDSTKVDKLVRDIYGGDYERFGLPGWAVASSFGNMMSKEKREAVSK EDLA


    Database link: Q9BZ23-1

  • Positive control

    • WB: Mouse liver and kidney tissue. IHC: Human colon cancer tissue. ICC/IF: HepG2 cells.

Properties

Applications

Our Abpromise guarantee covers the use of ab223091 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB 1/500 - 1/5000. Detects a band of approximately 31, 63 kDa (predicted molecular weight: 31, 63 kDa).
IHC-P 1/20 - 1/200.
ICC/IF 1/50 - 1/200.

Target

  • Function

    May be the master regulator of the CoA biosynthesis.
  • Tissue specificity

    Ubiquitous.
  • Pathway

    Cofactor biosynthesis; coenzyme A biosynthesis; CoA from (R)-pantothenate: step 1/5.
  • Involvement in disease

    Defects in PANK2 are the cause of neurodegeneration with brain iron accumulation type 1 (NBIA1) [MIM:234200]; also known as pantothenate kinase-associated neurodegeneration (PKAN) or Hallervorden-Spatz syndrome (HSS). It is an 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 the first decade, rapid progression, loss of independent ambulation within 15 years. Atypical forms have onset in the second decade, slow progression, maintenance of independent ambulation up to 40 years later. Intermediate forms manifest onset in the first decade with slow progression or onset in the 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.
    Defects in PANK2 are the cause of hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP) [MIM:607236]. HARP is a rare syndrome with many clinical similarities to NBIA1.
  • Sequence similarities

    Belongs to the type II pantothenate kinase family.
  • Cellular localization

    Cytoplasm and Mitochondrion.
  • Information by UniProt
  • Database links

  • Alternative names

    • 4933409I19Rik antibody
    • AI642621 antibody
    • C20orf48 antibody
    • Hallervorden Spatz syndrome antibody
    • HARP antibody
    • hPANK2 antibody
    • HSS antibody
    • MGC118448 antibody
    • MGC15053 antibody
    • mitochondrial antibody
    • NBIA1 antibody
    • PANK2 antibody
    • PANK2_HUMAN antibody
    • Pantothenate kinase 2 (Hallervorden Spatz syndrome) antibody
    • Pantothenate kinase 2 antibody
    • Pantothenate kinase 2 mitochondrial antibody
    • Pantothenic acid kinase 2 antibody
    • PKAN antibody
    • RP23 387C21.4 antibody
    see all

Images

  • Paraffin-embedded human colon cancer tissue stained for PANK2 using ab223091 at 1/100 dilution in immunohistochemical analysis.

  • All lanes : Anti-PANK2 antibody (ab223091) at 1/500 dilution

    Lane 1 : Mouse liver tissue lysate
    Lane 2 : Mouse kidney tissue lysate

    Secondary
    All lanes : Goat polyclonal to rabbit IgG at 1/50000 dilution

    Developed using the ECL technique.

    Predicted band size: 31, 63 kDa

  • PFA-fixed, Triton X-100 permeabilized HepG2 (human liver hepatocellular carcinoma cell line) cells stained for PANK2 (green) using ab223091 at 1/100 dilution in ICC/IF. Secondary: Alexa Fluor 488® conjugated Goat Anti-Rabbit IgG (H+L).

References

ab223091 has not yet been referenced specifically in any publications.

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