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蛋白质/抗原/多肽 >> 重组蛋白
重组肾病型胱氨酸症蛋白(CTNS)
重组肾病型胱氨酸症蛋白(CTNS)图片
交货期: 1周
索取资料及报价
交货期:1周
产品别名:重组胱氨酸蛋白
Recombinant CTNS
产品介绍
基因名:

CTNS


产品别名:

CTNS-LSB; PQLC4; SLC66A4; CTNS; cystinosin, lysosomal cystine transporter; cystinosin, lysosomal cystine transporter; cystinosin; cystinosis nephropathic; 肾病型胱氨酸症蛋白(CTNS); 胱氨酸;


背景信息:
Cystinosis is an autosomal recessive disorder resulting from defective lysosomal transport of cystine and present at birth as a failure to thrive, rickets and proximal renal tubular acidosis. The human CTNS gene on chromosome 17p13 encodes the protein Cystinosin, and mutations in CTNS are responsible for nephropathic cystinosis. The CTNS promoter contains an Sp1 binding element. Cystinosin is an integral membrane protein containing 7 transmembrane domains that functions as a H+-driven transporter responsible for cystine export from lysosomes. In humans, Cystinosin is expressed abundantly in pancreas, kidney (mature and fetal), and skeletal muscle. The mouse homolog to CTNS encodes a protein which is expressed in all tissues except skeletal muscle. In the cell, Cystinosin co-localizes with LAMP-2 to lysosomes. A C-terminal GYDQL sorting motif within Cystinosin is critical for lysosomal localization.

标签:His-tag   
分类:Recombinant  
类型:Protein   
偶联物:Unconjugated
内毒素水平:按批次,参阅瓶身标签
性状:Liquid
浓度:Batch dependent (Please refer to the vial label for the specific concentration.)
纯化类型:purified
内含物:不含防腐剂
应用:Positive Control;Immunogen;SDS-PAGE;WB.
储存:2-8°C不超过一个月,-80°C不超过12个月。避免反复冻融。
注意事项:仅供实验室使用。不适用于人类或动物的任何临床,治疗或诊断用途。不适合动物或人类食用。

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