Anti-CTNS Antibody |产品详情|进口榴莲视频免费观看采购网

您好，欢迎来到榴莲视频下载地址入口生物网站！请登录注册

我的订单 | 联系榴莲视频下载地址入口 | 在线客服QQ：3012681483 | 客服电话：400-968-7988 | 工作时间：08:30-17:30

主营产品： ELISA8008APP幸福宝榴莲、抗体、补体血清、兔补体、干扰素、小鼠测温仪、进口榴莲视频免费观看、Quidel代理、Swant代理、PBL Assay Science代理

网站首页产品中心代理品牌新闻资讯资源中心关于榴莲视频下载地址入口

所在位置：首页 > 产品中心 > Antibodies > Antibodies产品

订购信息

上海榴莲视频下载地址入口生物科技公司

Tel:400-968-7988 021-33779008

Anti-CTNS Antibody

品牌：Antibodies

货号：

规格：50µl

货期：

应用：

咨询客服

立即购买加入购物车

Anti-CTNS Antibody

商品详情参考文献相关资料

Overview

Name:	Anti-CTNS Antibody See all CTNS primary antibodies
Description:	Rabbit polyclonal antibody to CTNS.
Applications:	WB, IHC, IF
Dilutions:	WB: 1:500 - 1:2000, IHC: 1:50 - 1:200, IF: 1:50 - 1:100.
Reactivity:	Human, Mouse, Rat
Immunogen:	Recombinant protein of human CTNS.
Protein Length:	367 (400)
Host:	Rabbit
Clonality:	Polyclonal
Isotype:	IgG
Conjugate:	Unconjugated
Purification:	Affinity purification.
Product Form:	Liquid
Formulation:	Supplied in Phosphate Buffered Saline, pH 7.30, with 0.02% Sodium Azide and 50% Glycerol.
Storage:	Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

Target

Function:	Cystine/H(+) symporter thought to transport cystine out of lysosomes. Plays an important role in melanin synthesis, possibly by preventing melanosome acidification and subsequent degradation of tyrosinase TYR.
Tissue Specificity:	Strongly expressed in pancreas, kidney (*** and fetal), skeletal muscle, melanocytes and keratinocytes. Expressed at lower levels in placenta and heart. Weakly expressed in lung, liver and brain (*** and fetal). Isoform 2 represents 5-20 % of CTNS transcripts, with the exception of the testis that expresses both isoforms in equal proportions.
Involvement in Disease:	Cystinosis, nephropathic type: A form of cystinosis, a lysosomal storage disease due to defective transport of cystine across the lysosomal membrane. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. The classical nephropathic form has onset in the first year of life and is characterized by a polyuro-polydipsic syndrome, marked height-weight growth delay, generalized impaired proximal tubular reabsorptive capacity, with severe fluid-electrolyte balance alterations, renal failure, ocular symptoms and other systemic complications. Cystinosis, ***, non-nephropathic type: A form of cystinosis, a lysosomal storage disease due to defective transport of cystine across the lysosomal membrane. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. Cystinosis *** non-nephropathic type is characterized by ocular features and a benign course. Patients manifest mild photophobia due to conjunctival and corneal cystine crystals. Cystinosis, late-onset juvenile or adolescent nephropathic type: A form of cystinosis, a lysosomal storage disease due to defective transport of cystine across the lysosomal membrane. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. Late-onset juvenile or adolescent nephropathic cystinosis is an intermediated form, manifesting first at age 10 to 12 years with proteinuria due to glomerular damage rather than with the manifestations of tubular damage that occur first in infantile cystinosis. There is no excess amino aciduria and stature is normal. Photophobia, late development of pigmentary retinopathy, and chronic headaches are features.
Sequence Similarities:	Belongs to the cystinosin family.
Cellular locations:	Lysosome membrane. Melanosome.
Database Links:	Entrez Gene: 1497?Human Entrez Gene: 83429?Mouse Entrez Gene: 287478?Rat Omim: 606272?Human SwissProt: O60931?Human SwissProt: P57757?Mouse Unigene: 187667?Human Unigene: 259852?Mouse
Synonyms:	CTNS Antibody CTNS LSB Antibody CTNS_HUMAN Antibody Cystinosin Antibody Cystinosin, lysosomal cystine transporter Antibody cystinosis, nephropathic Antibody PQLC4 Antibody
Information:	Target information shown above is from the UniProt Consortium.