epithelial Sodium Channel alpha肽(ab4987)

概述

  • 产品名称epithelial Sodium Channel alpha肽

描述

  • 性质Synthetic

技术指标

Our Abpromise guarantee covers the use of ab4987 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 应用

    Blocking

  • 形式Liquid
  • 补充说明


    This peptide may be used for neutralization and control experiments with the polyclonal antibody that reacts with this product and endogenous alpha-ENaC, catalog ab3464. Using a solution of peptide of equal volume and concentration to the corresponding antibody will yield a large molar excess of peptide (~ 70-fold) for competitive inhibition of antibody-protein binding reactions.

  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

常规信息

  • 别名
    • Alpha ENaC
    • Alpha ENaC 2
    • Alpha NaCH
    • Alpha-ENaC
    • Alpha-NaCH
    • Amiloride sensitive epithelial sodium channel alpha subunit
    • Amiloride sensitive sodium channel subunit alpha
    • Amiloride-sensitive sodium channel subunit alpha
    • ENaCA
    • ENaCalpha
    • Epithelial Na(+) channel subunit alpha
    • Epithelial Na+ channel subunit alpha
    • FLJ21883
    • Nonvoltage gated sodium channel 1 subunit alpha
    • Nonvoltage-gated sodium channel 1 subunit alpha
    • SCNEA
    • SCNN 1
    • SCNN1
    • Scnn1a
    • SCNNA_HUMAN
    • Sodium channel nonvoltage gated 1 alpha
    see all
  • 功能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.
  • 组织特异性Highly expressed in kidney and lung. Detected at intermediate levels in pancreas and liver, and at low levels in heart and placenta. Isoform 1 and isoform 2 predominate in all tissues. Expression of isoform 3, isoform 4 and isoform 5 is very low or not detectable, except in lung and heart.
  • 疾病相关Defects in SCNN1A are a cause of autosomal recessive pseudohypoaldosteronism type 1 (AR-PHA1) [MIM:264350]. PHA1 is a rare salt wasting disease resulting from target organ unresponsiveness to mineralocorticoids. There are 2 forms of PHA1: the autosomal recessive form that is severe, and the dominant form which is milder and due to defects in mineralocorticoid receptor. AR-PHA1 is characterized by an often fulminant presentation in the neonatal period with dehydration, hyponatraemia, hyperkalaemia, metabolic acidosis, failure to thrive and weight loss. Note=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.
    Defects in SCNN1A are a cause of bronchiectasis with or without elevated sweat chloride type 2 (BESC2) [MIM:613021]; also called cystic fibrosis-like syndrome. BESC2 is 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 subnormal lung function, sinopulmonary infections, chronic productive cough, excessive sputum production, and elevated sweat chloride in some cases.
  • 序列相似性Belongs to the amiloride-sensitive sodium channel (TC 1.A.6) family. SCNN1A subfamily.
  • 翻译后修饰Ubiquitinated; this targets individual subunits for endocytosis and proteasome-mediated degradation.
  • 细胞定位Apical cell membrane. Apical membrane of epithelial cells.
  • Information by UniProt

epithelial Sodium Channel alpha peptide (ab4987)参考文献

ab4987 has not yet been referenced specifically in any publications.

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