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Upingbio
SKU:YP-Ab-05199-100UL
ERCC2 Polyclonal Antibody
ERCC2 Polyclonal Antibody
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- Gene Name: ERCC2 XPD XPDC
- Immunogen: Synthesized peptide derived from human protein . at AA range: 220-300
- Specificity: ERCC2 Polyclonal Antibody detects endogenous levels of protein.
- Composition: Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
- Source: Polyclonal, Rabbit,IgG
- Dilution ratio: WB 1:500-2000 ELISA 1:5000-20000
- Purification process: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
- Concentration: 1 mg/ml
- Storage: -20°C/1 year
- Background: The nucleotide excision repair pathway is a mechanism to repair damage to DNA. The protein encoded by this gene is involved in transcription-coupled nucleotide excision repair and is an integral member of the basal transcription factor BTF2/TFIIH complex. The gene product has ATP-dependent DNA helicase activity and belongs to the RAD3/XPD subfamily of helicases. Defects in this gene can result in three different disorders, the cancer-prone syndrome xeroderma pigmentosum complementation group D, trichothiodystrophy, and Cockayne syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Aug 2008],
- Function: cofactor:Magnesium.,disease:Defects in ERCC2 are a cause of trichothiodystrophy photosensitive (TTDP) [MIM:601675]. TTDP is an autosomal recessive disease characterized by sulfur-deficient brittle hair and nails, ichthyosis, mental retardation, impaired sexual development, abnormal facies and cutaneous photosensitivity correlated with a nucleotide excision repair (NER) defect. Neonates with trichothiodystrophy and ichthyosis are usually born with a collodion membrane. The severity of the ichthyosis after the membrane is shed is variable, ranging from a mild to severe lamellar ichthyotic phenotype. There are no reports of skin cancer associated with TTDP.,disease:Defects in ERCC2 are the cause of cerebro-oculo-facio-skeletal syndrome type 2 (COFS2) [MIM:610756]. COFS is a degenerative autosomal recessive disorder of prenatal onset affecting the brain, eye and spinal cord. After birth, it
- Species: Human;Mouse
- Range: WB;ELISA
- Protein: TFIIH basal transcription factor complex helicase XPD subunit (EC 3.6.4.12) (Basic transcription factor 2 80 kDa subunit) (BTF2 p80) (CXPD) (DNA excision repair protein ERCC-2) (DNA repair protein com
- DA: 83kD
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