DDB2 Recombinant Rabbit Monoclonal Antibody [JE16-41]

Function

Damaged DNA binding protein (DDB) is a heterodimer composed of two subunits, p127 and p48, which are designated DDB1 and DDB2, respectively. The DDB heterodimer is involved in repairing DNA damaged by ultraviolet light. Specifically, DDB, also designated UV-damaged DNA binding protein (UV-DDB), xeroderma pigmentosum group E binding factor (XPE-BF) and hepatitis B virus X-associated protein 1 (XAP-1), binds to damaged cyclobutane pyrimidine dimers (CPDs). Mutations in the DDB2 gene are implicated as causes of xeroderma pigmentosum group E, an autosomal recessive disease in which patients are defective in nucleotide excision DNA repair. XPE is characterized by hypersensitivity of the skin to sunlight with a high frequency of skin cancer as well as neurologic abnormalities. The hepatitis B virus (HBV) X protein interacts with DDB1, which may mediate HBx transactivation.

Background References

1. Inoki T et al. Human DDB2 splicing variants are dominant negative inhibitors of UV-damaged DNA repair. Biochem Biophys Res Commun 314:1036-1043 (2004).

2. Hwang B J et al. Expression of the p48 xeroderma pigmentosum gene is p53-dependent and is involved in global genomic repair. Proc Natl Acad Sci USA 96:424-428 (1999).

Sequence Similarity

Belongs to the WD repeat DDB2/WDR76 family.

Tissue Specificity

Ubiquitously expressed; with highest levels in corneal endothelium and lowest levels in brain. Isoform D1 is highly expressed in brain and heart. Isoform D2, isoform D3 and isoform D4 are weakly expressed.

Post-translational Modification

Phosphorylation by ABL1 negatively regulate UV-DDB activity.; Ubiquitinated by CUL4A in response to UV irradiation. Ubiquitination appears to both impair DNA-binding and promotes ubiquitin-dependent proteolysis. Degradation of DDB2 at sites of DNA damage may be a prerequisite for their recognition by XPC and subsequent repair. CUL4A-mediated degradation appears to be promoted by ABL1.; Ubiquitinated, leading to proteasomal degradation, and deubiquitinated by USP24.

Subcellular Location

Nucleus.

UNIPROT

SwissProt: Q92466 Human

Synonyms

damage-specific DNA binding protein 2 antibody

Damage-specific DNA-binding protein 2 antibody

DDB p48 subunit antibody

Ddb2 antibody

DDB2_HUMAN antibody

DDBb antibody

DNA damage-binding protein 2 antibody

UV-damaged DNA-binding protein 2 antibody

UV-DDB 2 antibody

Xeroderma pigmentosum group E protei antibody