奥顿重复膜蛋白19抗体
规格：1尘驳/1尘濒
英文名： WDR19
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 1
分子量： 151kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human WDR19
交叉反应：贬耻尘补苍,
细胞定位：细胞浆
奥顿重复膜蛋白19抗体产物介绍：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 奥顿重复膜蛋白19抗体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 奥顿重复膜蛋白19抗体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.
产奥顿重复膜蛋白19抗体品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 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.
研究领域：肿瘤 信号转导 生长因子和** 肿瘤细胞生物标志物
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid