Sacsin anticorps

N° du produit ABIN5708501

Détail du produit anti-Sacsin anticorps

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

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp Sacsin est non-conjugé

Application: ELISA, Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Purification: Antigen affinity purified

Immunogène: A recombinant human partial protein corresponding to amino acids E3709-L3909 was used as the immunogen for the Sacsin antibody.

Isotype: IgG

Information d'application

Indications d'application: Optimal dilution of the Sacsin antibody should be determined by the researcher.\. Western Blot: 0.5-1 μg/mL,IHC (FFPE): 1-2 μg/mL,Direct ELISA: 0.1-0.5 μg/mL

Restrictions: For Research Use only

Stockage

Buffer: 0.5 mg/mL if reconstituted with 0.2 mL sterile DI water

Stock: -20 °C

Stockage commentaire: After reconstitution, the Sacsin antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.

Détails sur Sacsin

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

Autre désignation: Sacsin (SACS Produits)

Synonymes: anticorps A230052M14, anticorps DNAJC29, anticorps E130115J16Rik, anticorps mKIAA0730, anticorps ARSACS, anticorps sacsin, anticorps sacsin molecular chaperone, anticorps Sacs, anticorps SACS

Sujet: Sacsin also known as DnaJ homolog subfamily C member 29 (DNAJC29) is a protein that in humans is encoded by the SACS gene. This gene consists of nine exons including a gigantic exon spanning more than 12.8k bp. It encodes the sacsin protein, which includes a UBQ region at the N-terminus, a HEPN domain at the C-terminus and a DnaJ region upstream of the HEPN domain. This modular protein is essential for normal mitochondrial network organization. The gene is highly expressed in the central nervous system, also found in skin, skeletal muscles and at low levels in the pancreas. Mutations in this gene result in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), a neurodegenerative disorder characterized by early-onset cerebellar ataxia with spasticityand peripheral neuropathy.

UniProt: Q9NZJ4