|Application ||WB, E|
|Calculated MW||41738 Da|
|Antigen Region||341-367 aa|
|Other Names||Cystinosin, CTNS|
|Target/Specificity||This CTNS antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 341-367 amino acids from the C-terminal region of human CTNS.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||CTNS Antibody(C-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
|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.|
|Cellular Location||Isoform 1: Lysosome membrane; Multi-pass membrane protein. Melanosome|
|Tissue Location||Strongly expressed in pancreas, kidney (adult and fetal), skeletal muscle, melanocytes and keratinocytes Expressed at lower levels in placenta and heart. Weakly expressed in lung, liver and brain (adult and fetal). Isoform 2 represents 5-20 % of CTNS transcripts, with the exception of the testis that expresses both isoforms in equal proportions|
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Provided below are standard protocols that you may find useful for product applications.
This gene encodes a seven-transmembrane domain protein that functions to transport cystine out of lysosomes. Its activity is driven by the H+ electrochemical gradient of the lysosomal membrane. Mutations in this gene cause cystinosis, a lysosomal storage disorder. Alternative splicing results in multiple transcript variants.
Taranta, A., et al. Pediatr. Nephrol. 25(7):1263-1267(2010)
Bellomo, F., et al. Free Radic. Biol. Med. 48(7):865-872(2010)
Kumar, A., et al. J. Biosci. 35(1):21-25(2010)
Aldahmesh, M.A., et al. Ophthalmic Genet. 30(4):185-189(2009)
Macias-Vidal, J., et al. Clin. Genet. 76(5):486-489(2009)
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