产品详情
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Product Name
CEP290 Rabbit pAb
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Gene ID
80184
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SwissProt ID
O15078
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Gene Name
CEP290
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Alternative Names
Centrosomal protein of 290 kDa; Cep290; Bardet-Biedl syndrome 14 protein; Cancer/testis antigen 87; CT87; Nephrocystin-6; Tumor antigen se2-2; CEP290; BBS14; KIAA0373; NPHP6
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Background
CEP290 activates ATF4 mediated transcription and is required for the correct localization of ciliary and phototransduction proteins in retinal photoreceptor cells; may play a role in ciliary transport processes. CEP290 is ubiquitously expressed; strongly in placenta and weakly in brain. There are two named isoforms. Defects in CEP290 are a cause of Joubert syndrome type 5 (JBTS5), Senior-Loken syndrome type 6 (SLSN6), Leber congenital amaurosis type 10 (LCA10) and Meckel syndrome type 4 (MKS4). Antibodies against CEP290 are present in sera from patients with cutaneous T cell lymphomas, but not in the healthy control population.
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Research Field
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Product Categories
Primary antibody
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Host
Rabbit
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Reactivity
Human,Mouse,Rat
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Application
WB
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Dilution Ratio
WB: 1/500-1/1000
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Molecular Weight
Calculated MW: 290kDa
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Clonality
Polyclonal Antibody
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Clonality No.
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Isotype
IgG
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Immunogen
Synthetic peptide corresponding to Human CEP290.
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Purification
Affinity Purified
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Conjugation
Unconjugated
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Modification
Unmodified
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Form
Liquid
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Buffer System
1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2.
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Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
应用缩写
WB:蛋白质印迹法IHC-P:石蜡切片样本的免疫组织化学法IHC-F:冰冻切片样本的免疫组织化学法ICC/IF:免疫细胞荧光 IP:免疫沉淀法FC:流式细胞术ChIP:染色质免疫沉淀法ELISA:酶联免疫吸附测定
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