Sacsin anticorps (AA 4291-4340)

N° du produit ABIN7231244

L’anticorps anti-Sacsin Polyclonal Lapin est utilisé pour la détection de Sacsin dans des échantillons de Humain et Souris. Il a été validé pour IHC et IF.

Aperçu rapide pour Sacsin anticorps (AA 4291-4340) (ABIN7231244)

Antigène: Sacsin (SACS) (Spastic Ataxia of Charlevoix-Saguenay (Sacsin) (SACS))

Reactivité: Humain, Souris

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp Sacsin est non-conjugé

Application: Immunohistochemistry (IHC), Immunofluorescence (IF)

Détail du produit anti-Sacsin anticorps

Épitope: AA 4291-4340

Fonction: Rabbit Anti-SACS Polyclonal Antibody

Specificité: The antibody detects endogenous levels of SACS protein

Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Immunogène: Synthesized peptide derived from part region of human SACS protein at AA range: 4291-4340

Isotype: IgG

Information d'application

Indications d'application: Optimal working dilutions should be determined experimentally by the investigator. Suggested starting dilutions are as follows: IHC 1:50-300,IF 1:50-200

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 mg/mL

Buffer: PBS, 50 % glycerol, 0.05 % Proclin 300, 0.05 %BSA

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: Stable for one year at -20°C from date of shipment. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Aliquot to avoid repeated freezing and thawing.

Date de péremption: 12 months

Détails sur Sacsin

Antigène: Sacsin (SACS) (Spastic Ataxia of Charlevoix-Saguenay (Sacsin) (SACS))

Autre désignation: SACS

Sujet: Sacsin, DnaJ homolog subfamily C member 29, DNAJC29SACS encodes the sacsin protein, which includes a UbL domain at the N-terminus, a DnaJ domain, and a HEPN domain at the C-terminus. SACS is highly expressed in the central nervous system, also found in skin, skeletal muscles and at low levels in the pancreas. SACS includes a very large exon spanning more than 12. kb. Mutations in SACS result in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), a neurodegenerative disorder characterized by early-onset cerebellar ataxia with spasticity and peripheral neuropathy. The authors of a publication on the effects of siRNA-mediated sacsin knockdown concluded that sacsin protects against mutant ataxin-1 and suggest that "the large multi-domain sacsin protein is able to recruit Hsp70 chaperone action and has the potential to regulate the effects of other ataxia proteins" (Parfitt et al. PubMed: 19208651). A pseudogene associated with this gene is located on chromosome 11. Alternative splicing of this gene results in multiple transcript variants.

Poids moléculaire: 503kD

ID gène: 26278

UniProt: Q9NZJ4