SKU: WM1238 Categories: ,

ERCC1 [PT0756] mouse mAb

Price range: $268.00 through $328.00

SKU: WM1238-50
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Product details

Background:
The product of this gene functions in the nucleotide excision repair pathway, and is required for the repair of DNA lesions such as those induced by UV light or formed by electrophilic compounds including cisplatin. The encoded protein forms a heterodimer with the XPF endonuclease (also known as ERCC4), and the heterodimeric endonuclease catalyzes the 5' incision in the process of excising the DNA lesion. The heterodimeric endonuclease is also involved in recombinational DNA repair and in the repair of inter-strand crosslinks. Mutations in this gene result in cerebrooculofacioskeletal syndrome, and polymorphisms that alter expression of this gene may play a role in carcinogenesis. Multiple transcript variants encoding different isoforms have been found for this gene. The last exon of this gene overlaps with the CD3e molecule, epsilon associated protein ge

Specifications

Size50μL/100μL
TargetERCC1
ReactivityHuman
ApplicationsWB, IF, ELISA
MW(Calculated)33kD
MW(Observed)33kD
Host SpeciesMouse
IsotypeIgG1,Kappa
Conjugate/ModificationUnmodified
Recommended Dilution RatioWB 1:500-2000; IF 1:100-500; ELISA 1:1000-5000
FormulationPBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
PurificationRecombinant Antibody expressed in animal component-free (ACF) media, purified via Protein G affinity chromatography.
Storage-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
ClonalityMonoclonal
Clone NumberRMM0756
ImmunogenSynthesized peptide derived from human ERCC1 AA range: 1-100
SpecificityThis antibody detects endogenous levels of ERCC1 protein.
Gene NameERCC1
Protein NameERCC1
Other NameDNA excision repair protein ERCC-1
SpeciesHuman
Gene ID2067
UniprotP07992
Species.1
Gene ID.1
Uniprot.1
Species.2
Gene ID.2
Uniprot.2
Cellular LocalizationNuclear
Tissue ExpressionCerebellum,Lung,Ovarian cancer,Uterus,
Signaling_pathway
Research Areas>>Platinum drug resistance; / >>Nucleotide excision repair; / >>Fanconi anemia pathway
FunctionDisease:Defects in ERCC1 are the cause of cerebro-oculo-facio-skeletal syndrome type 4 (COFS4) [MIM:610758]. 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.,Function:Structure-specific DNA repair endonuclease responsible for the 5'-incision during DNA repair.,similarity:Belongs to the ERCC1/RAD10/SWI10 family.,subunit:Heterodimer composed of ERCC1 and XPF/ERRC4.,
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