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        DDB2 Rabbit pAb (bs-2599R)  
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        產品編號 bs-2599R
        英文名稱 DDB2 Rabbit pAb
        中文名稱 DNA損傷結合蛋白2抗體
        別    名 damage-specific DNA binding protein 2; Damage-specific DNA-binding protein 2; DDB p48 subunit; Ddb2; DDB2_HUMAN; DDBb; DNA damage-binding protein 2; UV-damaged DNA-binding protein 2; UV-DDB 2.  
        研究領域 腫瘤  免疫學  染色質和核信號  轉錄調節因子  結合蛋白  表觀遺傳學  
        抗體來源 Rabbit
        克隆類型 Polyclonal
        克 隆 號
        交叉反應 Mouse,Rat (predicted: Human,Rabbit,Cow,Horse)
        產品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
        not yet tested in other applications.
        optimal dilutions/concentrations should be determined by the end user.
        理論分子量 48 kDa
        檢測分子量
        細胞定位 細胞核 
        性    狀 Liquid
        濃    度 1mg/ml
        免 疫 原 KLH conjugated synthetic peptide derived from human DDB2: 201-300/427 
        亞    型 IgG
        純化方法 affinity purified by Protein A
        緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
        保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
        注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
        PubMed PubMed
        產品介紹 This gene encodes a protein that is necessary for the repair of ultraviolet light-damaged DNA. This protein is the smaller subunit of a heterodimeric protein complex that participates in nucleotide excision repair, and this complex mediates the ubiquitylation of histones H3 and H4, which facilitates the cellular response to DNA damage. This subunit appears to be required for DNA binding. Mutations in this gene cause xeroderma pigmentosum complementation group E, a recessive disease that is characterized by an increased sensitivity to UV light and a high predisposition for skin cancer development, in some cases accompanied by neurological abnormalities. [provided by RefSeq].

        Function:
        Required for DNA repair. Binds to DDB1 to form the UV-damaged DNA-binding protein complex (the UV-DDB complex). The UV-DDB complex may recognize UV-induced DNA damage and recruit proteins of the nucleotide excision repair pathway (the NER pathway) to initiate DNA repair. The UV-DDB complex preferentially binds to cyclobutane pyrimidine dimers (CPD), 6-4 photoproducts (6-4 PP), apurinic sites and short mismatches. Also appears to function as the substrate recognition module for the DCX (DDB1-CUL4-X-box) E3 ubiquitin-protein ligase complex DDB1-CUL4-ROC1 (also known as CUL4-DDB-ROC1 and CUL4-DDB-RBX1). The DDB1-CUL4-ROC1 complex may ubiquitinate histone H2A, histone H3 and histone H4 at sites of UV-induced DNA damage. The ubiquitination of histones may facilitate their removal from the nucleosome and promote subsequent DNA repair. The DDB1-CUL4-ROC1 complex also ubiquitinates XPC, which may enhance DNA-binding by XPC and promote NER. Isoform D1 and isoform D2 inhibit UV-damaged DNA repair.

        Subunit:
        Component of the UV-DDB complex which includes DDB1 and DDB2. The UV-DDB complex interacts with monoubiquitinated histone H2A and binds to XPC via the DDB2 subunit. Component of the DCX (DDB1-CUL4-X-box) E3 ubiquitin-protein ligase complex DDB1-CUL4-ROC1 (also known as CUL4-DDB-ROC1 and CUL4-DDB-RBX1), which includes CUL4A or CUL4B, DDB1, DDB2 and RBX1. DDB2 may function as the substrate recognition module within this complex. The DDB1-CUL4-ROC1 complex may associate with the COP9 signalosome, and this inhibits the E3 ubiquitin-protein ligase activity of the complex. A large number of other DCX complexes may also exist in which an alternate substrate targeting subunit replaces DDB2. These targeting subunits are generally known as DCAF (DDB1- and CUL4-associated factor) or CDW (CUL4-DDB1-associated WD40-repeat) proteins. Isoform D1 and isoform D2 do not interact with DDB1.

        Subcellular Location:
        Nucleus. Note=Accumulates at sites of DNA damage following UV irradiation.

        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 modifications:
        Phosphorylation by ABL1 negatively regulate UV-DDB activity (By similarity).
        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.

        DISEASE:
        Xeroderma pigmentosum complementation group E (XP-E) [MIM:278740]: An autosomal recessive pigmentary skin disorder characterized by solar hypersensitivity of the skin, high predisposition for developing cancers on areas exposed to sunlight and, in some cases, neurological abnormalities. The skin develops marked freckling and other pigmentation abnormalities. XP-E patients show a mild phenotype with minimal or no neurologic features. Note=The disease is caused by mutations affecting the gene represented in this entry.

        Similarity:
        Belongs to the WD repeat DDB2/WDR76 family.
        Contains 7 WD repeats.

        SWISS:
        Q92466

        Gene ID:
        1643

        Database links:

        Entrez Gene: 519357 Cow

        Entrez Gene: 1643 Human

        Entrez Gene: 107986 Mouse

        Omim: 600811 Human

        SwissProt: Q0VBY8 Cow

        SwissProt: Q92466 Human

        SwissProt: Q99J79 Mouse

        Unigene: 446564 Human

        Unigene: 700338 Human

        Unigene: 260747 Mouse

        Unigene: 389334 Mouse



        產品圖片
        Sample: SP2/0(Human) Cell Lysate at 30 ug Primary: Anti- DDB2 (bs-2599R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 48 kD Observed band size: 48 kD
        Sample: Liver (Mouse) Lysate at 40 ug Primary: Anti- DDB2 (bs-2599R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 48 kD Observed band size: 48 kD
        Tissue/cell: Rat intestine tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-DDB2 Polyclonal Antibody, Unconjugated(bs-2599R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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