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Merck
CN

WH0001497M9

Monoclonal Anti-CTNS antibody produced in mouse

clone 5G6, purified immunoglobulin, buffered aqueous solution

Synonym(s):

Anti-CTNSLSB, Anti-PQLC4, Anti-cystinosis, nephropathic

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About This Item

NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
5G6, monoclonal
Application:
ELISA (i), WB
Citations:
3

Key Documents

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biological source

mouse

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

Quality Level

100

Gene Information

human ... CTNS(1497)

Related Categories

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.

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Storage Class

10 - Combustible liquids

flash_point_f

Not applicable

flash_point_c

Not applicable

ppe

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)

Regulatory Information

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Certificates of Analysis (COA)

Lot/Batch Number
07089-5G6

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Impact of cystinosin glycosylation on protein stability by differential dynamic SILAC
Nevo N, et al.
Molecular and Cellular Proteomics, 16, 457-468 (2017)
Molecular analysis of the CTNS gene in Jordanian families with nephropathic cystinosisAnalisis molecular del gen CTNS en familias jordanas afectadas por cistinosis nefropatica
Jaradat S, et al.
Nefrologia : Publicacion Oficial de la Sociedad Espa?ola Nefrologia, 35, 547-553 (2015)
First Report of CTNS Mutations in a Chinese Family with Infantile Cystinosis
Yang YJ, et al.
TheScientificWorldJournal (2015)

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