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货号： bs-6751R 基本售价： 1380.0 元 规格： 100ul
- 规格：100ul
- 价格：1380.00元
- 规格：200ul
- 价格：2200.00元
产品信息
- 产品编号
- bs-6751R
- 英文名称
- WDR19
- 中文名称
- 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/1380元购买 200ul/2200元购买 大包装/询价
- 说 明 书
- 100ul 200ul
- 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
- WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 151kDa
- 细胞定位
- 细胞浆
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human WDR19:101-200/1342
- 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.
- PubMed
- PubMed
- 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.
SWISS:
Q8NEZ3
Gene ID:
57728
Database links:Entrez Gene: 57728 Human
Entrez Gene: 213081 Mouse
Entrez Gene: 305349 Rat
Omim: 608151 Human
SwissProt: Q8NEZ3 Human
SwissProt: Q3UGF1 Mouse
Unigene: 438482 Human
Unigene: 127550 Mouse
Unigene: 18220 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.