Product nameXPD overexpression 293T lysate (whole cell)
DescriptionXPD overexpression lysate
ab94136 is a 293T cell transfected lysate in which Human XPD has been transiently over-expressed using a pCMV-XPD plasmid. The lysate is provided in 1X Sample Buffer. Note: For more details about how the transfected lysate was prepared view preparation notes
Tested applicationsSuitable for: WBmore details
Storage instructionsShipped on dry ice. Upon delivery aliquot and store at -20ºC. Avoid freeze / thaw cycles.
Storage bufferpH: 6.80
Constituents: 0.01% Bromophenol blue, 2.3% Beta mercaptoethanol, 2% Sodium lauryl sulfate, 0.788% Tris HCl, 10% Glycerol
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BackgroundFunction: ATP-dependent 5'-3' DNA helicase, component of the core-TFIIH basal transcription factor. Involved in nucleotide excision repair (NER) of DNA by opening DNA around the damage, and in RNA transcription by RNA polymerase II by anchoring the CDK-activating kinase (CAK) complex, composed of CDK7, cyclin H and MAT1, to the core-TFIIH complex. Involved in the regulation of vitamin-D receptor activity. As part of the mitotic spindle-associated MMXD complex it plays a role in chromosome segregation. Might have a role in aging process and could play a causative role in the generation of skin cancers. Disease: Defects in ERCC2 are the cause of xeroderma pigmentosum complementation group D (XP-D) [MIM:278730]; also known as XP group D (XPD). Xeroderma pigmentosum is 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. Some XP-D patients present features of Cockayne syndrome, including dwarfism, sensorineural deafness, microcephaly, mental retardation, pigmentary retinopathy, ataxia, decreased nerve conduction velocities. 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. 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 leads to brain atrophy, hypoplasia of the corpus callosum, hypotonia, cataracts, microcornea, optic atrophy, progressive joint contractures and growth failure. Facial dysmorphism is a constant feature. Abnormalities of the skull, eyes, limbs, heart and kidney also occur. Similarity: Belongs to the helicase family. RAD3/XPD subfamily. Contains 1 helicase ATP-binding domain. PTM: ISGylated.
Our Abpromise guarantee covers the use of ab94136 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use at an assay dependent dilution.|
ab94136 at 15µg/lane on an SDS-PAGE gel.
All lanes : Anti-XPD antibody (ab54676) at 1/500 dilution
Lane 1 :
XPD overexpression 293T lysate (whole cell) (ab94136)
Lane 2 : 293T non-transfected lysate
Lysates/proteins at 25 µg per lane.
All lanes : Goat Anti-mouse IgG (H and L) HRP conjugated at 1/2500 dilution
ab94136 has not yet been referenced specifically in any publications.