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.
