产品名称
ERCC1(1B10)Mouse Monoclonal Antibody
别名
ERCC1; DNA excision repair protein ERCC-1
蛋白名称
DNA excision repair protein ERCC-1
存储缓冲液
PBS, pH 7.4, containing 0.5%BSA, 0.02% New type preservative N as Preservative and 50% Glycerol.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=2067
Human Swissprot No.
P07992
Human Swissprot Link
http://www.uniprot.org/uniprotkb/P07992/entry
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=13870
Mouse Swissprot No.
P07903
Mouse Swissprot Link
http://www.uniprot.org/uniprot/P07903
Rat Swissprot Link
http://www.uniprot.org/uniprot/
免疫原
Synthetic Peptide of ERCC1
特异性
The antibody detects endogenous ERCC1 proteins.
稀释度
IHC-p: 100-300.WB 1:1000. IF 1:50-200
背景介绍
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
组织表达
Cerebellum,Lung,Ovarian cancer,Uterus,
细胞定位
[Isoform 1]: Nucleus .; [Isoform 2]: Cytoplasm . Nucleus .; [Isoform 3]: Nucleus .; [Isoform 4]: Nucleus .
信号通路
Nucleotide excision repair;
功能
disease: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.,
纯化
The antibody was affinity-purified from mouse ascites by affinity-chromatography using specific immunogen.
