Anti-WDR19/FITC Conjugated抗体-抗体-抗体-生物在线
Anti-WDR19/FITC Conjugated抗体

Anti-WDR19/FITC Conjugated抗体

商家询价

产品名称： Anti-WDR19/FITC Conjugated抗体

英文名称： Anti-WDR19/FITC

产品编号： YB--6751R-FITC

产品价格： null

产品产地： 中国/美国

品牌商标： Ybscience

更新时间： 2023-08-17T10:29:50

使用范围： 科研使用

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 Anti-WDR19/FITC Conjugated抗体

产品编号 YB-6751R-FITC
英文名称 Anti-WDR19/FITC
中文名称 FITC标记的WD重复膜蛋白19抗体
别    名 DYF 2; DYF2; Intraflagellar transport 144 homolog; KIAA1638; ORF26; Oseg6; PWDMP; WD repeat containing protein 19; WD repeat domain 19; WD repeat membrane protein PWDMP; WD repeat-containing protein 19; Wdr19; WDR19_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  信号转导  生长因子和激素  肿瘤细胞生物标志物  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, 
产品应用 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 151kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human WDR19
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
May be involved in cilia function and/or assembly. Tissue specificity: Some isoforms are tissue-specific. Highly expressed in the prostate. Lower expression in the cerebellum, pituitary gland, fetal lung, and pancreas. In normal prostate, expressed in both basal and luminal epithelial cells. No expression detected in fibromuscular stromal cells, endothelial cells, or infiltrating lymphocytes. Uniformed expression in prostate adenocarcinoma cells.

Function:
May be involved in cilia function and/or assembly. 

Subcellular Location:
Cell projection, cilium (By similarity). Cytoplasm, cytoskeleton, cilium basal body (By similarity). Note=Localizes to photoreceptor connecting cilia, to the base of motile cilia in brain ependymal cells and to the base of and along primary cilia in kidney cells (By similarity).

Tissue Specificity:
Some isoforms are tissue-specific. Highly expressed in the prostate. Lower expression in the cerebellum, pituitary gland, fetal lung, and pancreas. In normal prostate, expressed in both basal and luminal epithelial cells. No expression detected in fibromuscular stromal cells, endothelial cells, or infiltrating lymphocytes. Uniformed expression in prostate adenocarcinoma cells.

DISEASE:
Defects in WDR19 are the cause of cranioectodermal dysplasia type 4 (CED4) [MIM:614378]. CED4 is a disorder primarily characterized by craniofacial, skeletal and ectodermal abnormalities. Clinical features include craniosynostosis, narrow rib cage, short limbs, brachydactyly, hypoplastic and widely spaced teeth, sparse hair, skin laxity and abnormal nails. Nephronophthisis leading to progressive renal failure, hepatic fibrosis, heart defects, and retinitis pigmentosa have also been described.
Defects in WDR19 are the cause of asphyxiating thoracic dystrophy type 5 (ATD5) [MIM:614376]. ATD5 is an autosomal recessive chondrodysplasia characterized by a severely constricted thoracic cage, short-limbed short stature, and polydactyly. It often leads to death in infancy because of respiratory insufficiency. Retinal degeneration, cystic renal disease and hepatic disease can be present in affected individuals who survive early childhood.
Defects in WDR19 are the cause of nephronophthisis type 13 (NPHP13) [MIM:614377]. NPHP13 is an autosomal recessive disorder resulting in end-stage renal disease. It is a progressive tubulo-interstitial kidney disorder histologically characterized by modifications of the tubules with thickening of the basement membrane, interstitial fibrosis and, in the advanced stages, medullary cysts. 

Similarity:
Contains 6 TPR repeats.
Contains 6 WD repeats. 

Database links:
UniProtKB/Swiss-Prot: Q8NEZ3.2

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications