產品詳情
簡單介紹：
Anti-CTNS抗體產品質量穩定，實驗效果明顯，貨期快，價格優惠，歡迎垂詢訂購！我公司長期供應**組化抗體、WB抗體、**組化試劑盒和抗體試驗所需全部相關試劑、熒光標記抗體、單克隆抗體、多克隆抗體、各種標記的二抗IgG/IgM/IgD/IgA等科研實驗抗體。Anti-CTNS抗體用于**組化實驗,WB實驗,相應的標記抗體有HRP標記抗體,FITC標記,BIO等。
詳情介紹：
Rabbit Anti-CTNS
Cat. Number:
Anti-CTNS抗體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抗體 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抗體 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抗體 -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抗體 dilutions must be determined by the end user.
Not yet tested in other applications.
Optimal workingAnti-CTNS抗體 dilutions must be determined by the end user.
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