Anti-PANK2 antibody (bs-8338R)

Anti-PANK2 antibody

DATASHEET

Host:Rabbit

Target Protein:PANK2

IR:Immunogen Range:401-500/570

Clonality:Polyclonal

Isotype:IgG

Entrez Gene:80025

Swiss Prot:Q9BZ23

Source:KLH conjugated synthetic peptide derived from human PANK2:401-500/570 

Purification:affinity purified by Protein A

Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

Background:Defects in PANK2 are the cause of neurodegeneration with brain iron accumulation type 1 (NBIA1); 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). HARP is a rare syndrome with many clinical similarities to NBIA1.

Size:100ul

Concentration:1mg/ml

Applications:WB(1:500-2000)
ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
IF(1:50-200)

Cross Reactive Species:Human
Mouse
Rat
Dog
Pig
Cow
Horse
.

For research use only. Not intended for diagnostic or therapeutic use.

VALIDATION IMAGES

Sample:
Lane 1: HepG2 (Human) Cell Lysate at 30 ug
Lane 2: U251 (Human) Cell Lysate at 30 ug
Primary: Anti-PANK2 (bs-8338R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 62/50 kD
Observed band size: 62 kD

Sample:
Bone (Mouse) Lysate at 40 ug
Primary: Anti-PANK2 (bs-8338R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 57 kD
Observed band size: 57 kD