168142

Rabbit Anti-DDB1 (DNA Damage-binding Protein 1, DDB p127 Subunit, DNA Damage-binding Protein a, DDBa, Damage-specific DNA-binding Protein 1, HBV X-associated Protein 1, XAP-1, UV-damaged DNA-binding Factor, UV-damaged DNA-binding Protein 1, UV-DDB 1, XPE-binding Factor, XPE-BF, Xeroderma pigmentosum Group E-complementing Protein, XPCe, XAP1) (Not for Export EU)

Damage specific DNA binding protein 1 antibody, DDB 1 antibody, DDB p127 subunit antibody, DDBa antibody, DNA damage binding protein 1 antibody, UV damaged DNA binding factor antibody

DDB1 is also known as damage-specific DNA binding protein 1, DDB p127 subunit, DDBa, UV-damaged DNA-binding protein 1, UV-DDB 1, Xeroderma pigmentosum group E complementing protein, XPCe, X-associated protein 1 and XAP-1. The DDB1 gene encodes the large subunit (p127) of DNA damage-binding protein, which is a heterodimer, composed of a large and a small subunit (p48 DDB2). This nuclear protein functions in nucleotide-excision repair resulting from UV-damaged DNA by binding to pyrimidine dimers. Its defective activity causes the repair defect in the patients with xeroderma pigmentosum complementation group E (XPE). XP-E is a rare human autosomal recessive disease characterized by solar sensitivity, high predisposition for developing cancers on areas exposed to sunlight and, in some cases, neurological abnormalities. However, it remains for mutation analysis to demonstrate whether the defect in XPE patients is in this gene or the gene encoding the small subunit. In addition, Best vitelliform mascular dystrophy is mapped to the same region as this gene on 11q, but no sequence alternations of this gene are demonstrated in best disease patients.