SAB5702646

Anti-DLAT Antibody, clone 2Z7R10, Rabbit Monoclonal

• Synonyme(s) : DLTA, PDC-E2, PDCE2
• Code UNSPSC : 12352203
• Nomenclature NACRES : NA.46

Propriétés

• Source biologique: rabbit
• Niveau de qualité: 100
• Matériaux: colorless
• Clone: 2Z7R10, monoclonal
• Forme: liquid
• Poids mol.: 70 kDa
• Espèces réactives: rat, rat, human
• Concentration: 0.24 mg/mL
• Technique(s): western blot: 1:500 - 1:2000
• Couleur: colorless
• Isotype: IgG
• Séquence immunogène: AREGKLQPHEFQGGTFTISNLGMFGIKNFSAIINPPQACILAIGASEDKLVPADNEKGFDVASMMSVTLSCDHRVVDGAVGAQWLAEFRKYLEKPITMLL
• Numéro d'accès UniProt: P10515
• Conditions d'expédition: wet ice
• Température de stockage: −20°C
• Modification post-traductionnelle de la cible: unmodified
• Informations sur le gène: human ... DLAT(1737)

Description

Description générale

This gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC resides in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, accepts acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC enventually leads to cirrhosis and liver failure. Mutations in this gene are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lactic acidosis in infancy and early childhood.[provided by RefSeq, Oct 2009]

Immunogène

A synthetic peptide corresponding to a sequence within amino acids 548-647 of human DLAT (P10515).

PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.

Application

WB

Informations sur la sécurité

• Code de la classe de stockage: 10 - Combustible liquids
• Classe de danger pour l'eau (WGK): WGK 1