产品详情
简单介绍：
Anti-CTNS抗体产品质量稳定，实验效果明显，货期快，价格优惠，欢迎垂询订购！我公司长期供应**组化抗体、WB抗体、**组化试剂盒和抗体试验所需全部相关试剂、荧光标记抗体、单克隆抗体、多克隆抗体、各种标记的二抗IgG/IgM/IgD/IgA等科研实验抗体。Anti-CTNS抗体用于**组化实验,WB实验,相应的标记抗体有HRP标记抗体,FITC标记,BIO等。
详情介绍：
Rabbit Anti-CTNS
Cat. Number:
Anti-CTNS抗体KL-12932R
Quantity size:
0.2ml
Concentration:
1mg/ml Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
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 functionsAnti-CTNS抗体 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.
Also known as:
CTNS LSB; Cystinosin;Anti-CTNS抗体 cystinosis, nephropathic; PQLC4; CTNS_HUMAN.
Specificity:
●
Rabbit Polyclonal IgG, affinity purified by Protein A.
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Reacts with: Human, Mouse, Rat, Rabbit, .
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Immunogen: KLH conjugated synthetic peptide derived from human CTNS/Cystinosin.
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Predicted Molecular Weight: 42kDa.
Storage:
Shipped at 4℃, Store atAnti-CTNS抗体 -20℃ (Avoid repeated freeze/thaw cycles).
Application:
ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500
Not yet tested in other applications.
Optimal workingAnti-CTNS抗体 dilutions must be determined by the end user.
Not yet tested in other applications.
Optimal workingAnti-CTNS抗体 dilutions must be determined by the end user.
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