FGF-2 Polyclonal Antibody
¥680.00
- 产品信息
商品描述
靶点-Target
ERCC1
反应性
Human,Mouse,Rat
应用
WB,IHC,IF,ELISA
宿主物种-Host Species
Rabbit
同种型-Isotype
IgG,Kappa
MW(Calculated)
33kD
MW(Observed)
39kD
推荐稀释比
IHC 1:200-1:1000;WB 1:2000-1:10000;IF 1:200-1:1000;ELISA 1:5000-1:20000;
组成
PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
纯化工艺
Protein A
储存
-15°C to -25°C/1 year(Do not lower than -25°C)
克隆性
Monoclonal
克隆号
PT1303R
特异性
Endogenous
基因名称
ERCC1
蛋白名称
DNA excision repair protein ERCC-1
别名
ERCC1;DNA excision repair protein ERCC-1
Organism-1
Human
基因ID-1
2067
SwissProt-1
P07992
Organism-2
Mouse
基因ID-2
13870
SwissProt-2
P07903
背景
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
细胞定位
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.,
