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Cat. Number:

bs-12932R-Gold

Quantity size:

100ul   10nm

Concentration:

0.4mg/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 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.

Also known as:

CTNS LSB; Cystinosin; cystinosis, nephropathic; PQLC4; CTNS_HUMAN.

Specificity:

Storage:

0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300. Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.

Application:

Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.

Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.