84-4366-49　DDB1 (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) 100ug　350592

特徴

• The protein encoded by this gene is the large subunit (p127) of the heterodimeric DNA damage-binding (DDB) complex while another protein (p48) forms the small subunit.
• And this protein complex functions in nucleotide-excision repair and binds to DNA following UV damage.
• Defective activity of this complex causes the repair defect in patients with xeroderma pigmentosum complementation group E (XPE) - an autosomal recessive disorder characterized by photosensitivity and early onset of carcinomas.
• 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.
• The protein encoded by this gene also functions as an adaptor molecule for the cullin 4 (CUL4) ubiquitin E3 ligase complex by facilitating the binding of substrates to this complex and the ubiquitination of proteins.
• Applications:Suitable for use in Western Blot, Immunohistochemistry.
• Other applications not tested.
• Recommended Dilution:Western Blot: 0.1-0.5ug/ml Immunohistochemistry (paraffin): 0.5-1ug/ml Optimal dilutions to be determined by the researcher.
• Storage and Stability:May be stored at 4°C for short-term only.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20°C.
• Aliquots are stable for 12 months.
• For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.