Monoclonal Anti-CTNS antibody produced in mouse

The cystinosin, lysosomal cystine transporter (CTNS) gene with 12 exons, spanning 23kb on genomic DNA, is mapped to human chromosome 17p13.3. The cystinosin protein contains seven-transmembrane-domains and is mainly localized in lysosomes.

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. (provided by RefSeq)

CTNS (NP_004928, 1 a.a. ~ 100 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Sequence
MIRNWLTIFILFPLKLVEKCESSVSLTVPPVVKLENGSSTNVSLTLRPPLNATLVITFEITFRSKNITILELPDEVVVPPGVTNSSFQVTSQNVGQLTVY

Cystinosin (CTNS) plays a vital role in migrating cystine out of lysosomes. Mutations in CTNS gene is associated with the development of cystinosis, an autosomal recessive disease characterized by intralysosomal accumulation of cystine.

Solution in phosphate buffered saline, pH 7.4

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