PANK2 anticorps (AA 401-500) (AbBy Fluor® 594)

N° du produit ABIN2805063

Détail du produit anti-PANK2 anticorps (AbBy Fluor® 594)

Antigène: PANK2 (Pantothenate Kinase 2 (PANK2))

Épitope: AA 401-500

Reactivité: Souris

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp PANK2 est conjugé à/à la AbBy Fluor® 594

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

Réactivité croisée: Souris

Homologie: Human,Rat,Dog,Cow,Pig,Horse

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human PANK2

Isotype: IgG

Information d'application

Indications d'application: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Stockage

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.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Stock: -20 °C

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

Date de péremption: 12 months

Détails sur PANK2

Antigène: PANK2 (Pantothenate Kinase 2 (PANK2))

Autre désignation: PANK2 (PANK2 Produits)

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

Sujet:

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.

ID gène: 80025

UniProt: Q9BZ23

Pathways: Ribonucleoside Biosynthetic Process