PANK2 antibody (AA 401-500) (AbBy Fluor® 750)

Catalog No. ABIN5008316

Product Details anti-PANK2 Antibody (AbBy Fluor® 750)

Target: PANK2 (Pantothenate Kinase 2 (PANK2))

Binding Specificity: AA 401-500

Reactivity: Mouse

Host: Rabbit

Clonality: Polyclonal

Conjugate: This PANK2 antibody is conjugated to AbBy Fluor® 750

Application: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Cross-Reactivity: Mouse

Predicted Reactivity: Human,Rat,Dog,Cow,Pig,Horse

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human PANK2

Isotype: IgG

Application Details

Application Notes: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for PANK2

Target: PANK2 (Pantothenate Kinase 2 (PANK2))

Alternative Name: PANK2 (PANK2 Products)

Synonyms: ATPANK2 antibody, F10M6.180 antibody, F10M6_180 antibody, pantothenate kinase 2 antibody, PANK2 antibody, C20orf48 antibody, HARP antibody, HSS antibody, NBIA1 antibody, PKAN antibody, 4933409I19Rik antibody, AI642621 antibody, pantothenate kinase 2 antibody, pantothenate kinase 2, mitochondrial antibody, ribonuclease A family member 11 (inactive) antibody, PANK2 antibody, Tsp_01576 antibody, LOC100539295 antibody, Pank2 antibody, RNASE11 antibody

Background:

Synonyms: Pantothenate kinase 2, mitochondrial, hPanK2, Pantothenic acid kinase 2, PANK2, C20orf48, PANK2_HUMAN

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.

Gene ID: 80025

UniProt: Q9BZ23

Pathways: Ribonucleoside Biosynthetic Process