产品资料

Anti-CLCN5 Antibody

如果您对该产品感兴趣的话,可以
产品名称: Anti-CLCN5 Antibody
产品型号:
产品展商: 其它品牌
产品文档: 无相关文档

简单介绍

Anti-CLCN5 Antibody


Anti-CLCN5 Antibody  的详细介绍
Name: Anti-CLCN5 Antibody
See all CLCN5 primary antibodies
Description: Rabbit polyclonal antibody to CLCN5
Specificity: CLCN5 pAb detects endogenous levels of CLCN5 protein.
Applications: WB, IHC
Reactivity: Human, Mouse, Rat
Immunogen: Recombinant full length Human CLCN5.
Host: Rabbit
Clonality: Polyclonal
Conjugate: Unconjugated
Molecular Weight: ~83 kDa
Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Product Form: 1mg/ml in PBS?with?0.1%?Sodium?Azide,?50%?Glycerol.
Function: Proton-coupled chloride transporter. Functions as antiport system and exchanges chloride ions against protons. Important for normal acidification of the endosome lumen. May play an important role in renal tubular function.
Tissue Specificity: Kidney. Moderately expressed in aortic vascular smooth muscle and endothelial cells, and at a slightly higher level in the coronary vascular smooth muscle.
Involvement in Disease: Hypophosphatemic rickets, X-linked recessive: A renal disease belonging to the 'Dent disease complex', a group of disorders characterized by proximal renal tubular defect, hypercalciuria, nephrocalcinosis, and renal insufficiency. The spectrum of phenotypic features is remarkably similar in the various disorders, except for differences in the severity of bone deformities and renal impairment. XLRH patients present with rickets or osteomalacia, hypophosphatemia due to decreased renal tubular phosphate reabsorption, hypercalciuria, and low molecular weight proteinuria. Patients develop nephrocalcinosis with progressive renal failure in *****hood. Female carriers may have asymptomatic hypercalciuria or hypophosphatemia only.

Nephrolithiasis 2: An X-linked recessive renal disease belonging to the 'Dent disease complex', a group of disorders characterized by proximal renal tubular defect, hypercalciuria, nephrocalcinosis, and renal insufficiency. The spectrum of phenotypic features is remarkably similar in the various disorders, except for differences in the severity of bone deformities and renal impairment. Nephrolithiasis type 2 patients manifest hypercalciuria, hypophosphatemia, aminoaciduria, nephrocalcinosis and nephrolithiasis, renal insufficiency leading to renal failure in *****hood, rickets (33% of patients) and osteomalacia.

Nephrolithiasis 1: An X-linked recessive renal disease belonging to the 'Dent disease complex', a group of disorders characterized by proximal renal tubular defect, hypercalciuria, nephrocalcinosis and renal insufficiency. The spectrum of phenotypic features is remarkably similar in the various disorders, except for differences in the severity of bone deformities and renal impairment. Nephrolithiasis type 1 presents with hypercalciuria, nephrocalcinosis, renal stones and renal insufficiency. Patients lack urinary acidification defects, rickets, and osteomalacia.

Low molecular weight proteinuria with hypercalciuria and nephrocalcinosis: An X-linked renal disease belonging to the 'Dent disease complex', a group of disorders characterized by proximal renal tubular defect, hypercalciuria, nephrocalcinosis, and renal insufficiency. The spectrum of phenotypic features is remarkably similar in the various disorders, except for differences in the severity of bone deformities and renal impairment. LMWPHN is a slowly progressive disorder. Patients tend to have hypercalciuric nephrocalcinosis without rickets or renal failure.
Sequence Similarities: Belongs to the chloride channel (TC 2.A.49) family. ClC-5/CLCN5 subfamily.
Post-Translational Modification: Ubiquitinated by NEDD4L in the presence of albumin; which promotes endocytosis and proteasomal degradation.
Cellular Location: Golgi apparatus membrane. Endosome membrane. Cell membrane.
Database Links:
  • Entrez Gene: 1184?Human
  • Entrez Gene: 12728?Mouse
  • Entrez Gene: 25749?Rat
  • Omim: 300008?Human
  • SwissProt: P51795?Human
  • SwissProt: Q9WVD4?Mouse
  • SwissProt: P51796?Rat
  • Unigene: 166486?Human
  • Unigene: 745501?Human
  • Unigene: 486257?Mouse
  • Unigene: 10337?Rat
    		•
    Synonyms:
  • Chloride Channel 5 Antibody
  • Chloride channel protein 5 Antibody
  • Chloride channel voltage sensitive 5 Antibody
  • Chloride transporter ClC-5 Antibody
  • ClC-5 Antibody
  • CLC5 Antibody
  • CLCK2 Antibody
  • CLCN5 Antibody
  • CLCN5_HUMAN Antibody
  • DENTS Antibody
  • H(+)/Cl(-) exchange transporter 5 Antibody
  • hCIC-K2 Antibody
  • NPHL1 Antibody
  • NPHL2 Antibody
  • Voltage gated chloride ion channel CLCN5 Antibody
  • XLRH Antibody
  • XRN Antibody
    		•
    Information: Target information shown above is from the UniProt Consortium.
    产品留言
    标题
    联系人
    联系电话
    内容
    验证码
    点击换一张
    注：1.可以使用快捷键Alt+S或Ctrl+Enter发送信息!
    2.如有必要,请您留下您的详细联系方式!

    沪公网安备 31011202007337号