扫码关注公众号           扫码咨询技术支持           扫码咨询技术服务
  
客服热线:400-901-9800  客服QQ:4009019800  技术答疑  技术支持  质量反馈  人才招聘  关于我们  联系我们
产品中心-北京博奥森生物技术有限公司
首页 > 产品中心 > 标记一抗 > 产品信息
Rabbit Anti-phospho-SCNN1B(Ser633) /Gold Conjugated antibody (bs-5702R-Gold)
订购热线:400-901-9800
订购邮箱:sales@bioss.com.cn
订购QQ:  400-901-9800
技术支持:techsupport@bioss.com.cn
说 明 书: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包装/询价
产品编号 bs-5702R-Gold
英文名称 Anti-phospho-SCNN1B(Ser633) /Gold
中文名称 胶体金标记的磷酸化上皮钠通β抗体
别    名 SCNN1B(phospho S633); Amiloride sensitive sodium channel subunit beta; Beta NaCH; ENaC beta; ENaCB; Epithelial Na(+) channel subunit beta; Epithelial Na+ channel beta subunit; Epithelial Na+ channel subunit beta; Epithelial sodium channel beta 2 subunit; Epithelial sodium channel beta 3 subunit; Nonvoltage gated sodium channel 1 beta subunit; Nonvoltage gated sodium channel 1 subunit beta; SCNEB; SCNN 1B; SCNNB_HUMAN; Sodium channel nonvoltage gated 1 beta (Liddle syndrome); Sodium channel nonvoltage gated 1 beta; SCNNB_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul(10nm  15nm  35nm
产品类型 磷酸化抗体 
研究领域 肿瘤  免疫学  神经生物学  细胞膜受体  新陈代谢  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse,  (predicted: Rat, Dog, Pig, Cow, Guinea Pig, G)
产品应用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 73kDa
性    状 Lyophilized or Liquid
浓    度 0.4mg/ml
免 疫 原 KLH conjugated Synthesised phosphopeptide derived from human SCNN1B around the phosphorylation site of Ser633
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存条件 Store at 2-8 ºC for 3-6 months. Avoid repeated freeze/thaw cycles.
产品介绍 background:
SCNN1B is a subunit of the epithelial sodium channel, ENaC. ENac has high sodium selectivity, low conductance, and amiloride sensitivity. The functional channel of ENaC is composed of at least 3 subunits, alpha (SCNN1A), beta (SCNN1B), and gamma (SCNN1G). The 3 subunits show sequence similarities to one another, indicating descent from a common ancestral gene. Each encodes a protein containing 2 transmembrane domains, with intracellular amino and carboxyl termini.

Function:
Sodium permeable non-voltage-sensitive ion channel inhibited by the diuretic amiloride. Mediates the electrodiffusion of the luminal sodium (and water, which follows osmotically) through the apical membrane of epithelial cells. Controls the reabsorption of sodium in kidney, colon, lung and sweat glands. Also plays a role in taste perception.

Subunit:
Probable heterotrimer containing one alpha, one beta and one gamma subunit. A delta subunit can replace the alpha subunit. Interacts with the WW domains of NEDD4, NEDD4L, WWP1 and WWP2. Interacts with the full length immature form of PCSK9 (pro-PCSK9).

Subcellular Location:
Apical cell membrane; Multi-pass membrane protein. Note=Apical membrane of epithelial cells.

Post-translational modifications:
Phosphorylated on serine and threonine residues (By similarity).

DISEASE:
Pseudohypoaldosteronism 1, autosomal recessive (PHA1B) [MIM:264350]: A rare salt wasting disease resulting from target organ unresponsiveness to mineralocorticoids. PHA1B is a severe form involving multiple organ systems, and characterized by an often fulminant presentation in the neonatal period with dehydration, hyponatremia, hyperkalemia, metabolic acidosis, failure to thrive and weight loss. Note=The disease is caused by mutations affecting the gene represented in this entry. The degree of channel function impairment differentially affects the renin-aldosterone system and urinary Na/K ratios, resulting in distinct genotype-phenotype relationships in PHA1 patients. Loss-of-function mutations are associated with a severe clinical course and age-dependent hyperactivation of the renin-aldosterone system. This feature is not observed in patients with missense mutations that reduce but do not eliminate channel function. Markedly reduced channel activity results in impaired linear growth and delayed puberty (PubMed:18634878).
Liddle syndrome (LIDDS) [MIM:177200]: Autosomal dominant disorder characterized by pseudoaldosteronism and hypertension associated with hypokalemic alkalosis. The disease is caused by constitutive activation of the renal epithelial sodium channel. Note=The disease is caused by mutations affecting the gene represented in this entry.
Bronchiectasis with or without elevated sweat chloride 1 (BESC1) [MIM:211400]: A debilitating respiratory disease characterized by chronic, abnormal dilatation of the bronchi and other cystic fibrosis-like symptoms in the absence of known causes of bronchiectasis (cystic fibrosis, autoimmune diseases, ciliary dyskinesia, common variable immunodeficiency, foreign body obstruction). Clinical features include sub-normal lung function, sinopulmonary infections, chronic productive cough, excessive sputum production, and elevated sweat chloride in some cases. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the amiloride-sensitive sodium channel (TC 1.A.6) family. SCNN1B subfamily.

Database links:

Entrez Gene: 6338 Human

Entrez Gene: 20277 Mouse

Entrez Gene: 24767 Rat

Omim: 600760 Human

SwissProt: P51168 Human

SwissProt: Q9WU38 Mouse

SwissProt: P37090 Rat

Unigene: 414614 Human

Unigene: 7709 Mouse

Unigene: 9807 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版权所有 2004-2026 www.bioss.com.cn 北京博奥森生物技术有限公司
通过国际质量管理体系ISO 9001:2015 GB/T 19001-2016    认证编号: 00122Q31509R1M/1100
京ICP备05066980号-1         京公网安备110107000727号