WH0001497M9

Monoclonal Anti-CTNS antibody produced in mouse

clone 5G6, purified immunoglobulin, buffered aqueous solution

• Synonym(s): Anti-CTNSLSB, Anti-PQLC4, Anti-cystinosis, nephropathic
• UNSPSC Code: 12352203
• NACRES: NA.41

Properties

• biological source: mouse
• Quality Level: 100
• conjugate: unconjugated
• antibody form: purified immunoglobulin
• antibody product type: primary antibodies
• clone: 5G6, monoclonal
• form: buffered aqueous solution
• species reactivity: human
• technique(s): indirect ELISA: suitable; western blot: 1-5 μg/mL
• isotype: IgG2aκ
• GenBank accession no.: NM_004937
• UniProt accession no.: O60931
• shipped in: dry ice
• storage temp.: −20°C
• target post-translational modification: unmodified
• Gene Information: human ... CTNS(1497)

Description

General description

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)

Immunogen

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

Biochem/physiol Actions

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.

Physical form

Solution in phosphate buffered saline, pH 7.4

Legal Information

GenBank is a registered trademark of United States Department of Health and Human Services

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Safety Information

• Storage Class: 10 - Combustible liquids
• flash_point_f: Not applicable
• flash_point_c: Not applicable
• ppe: Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)