WH0001497M9
Monoclonal Anti-CTNS antibody produced in mouse
clone 5G6, purified immunoglobulin, buffered aqueous solution
别名:
Anti-CTNSLSB, Anti-PQLC4, Anti-cystinosis, nephropathic
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关于此项目
NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
5G6, monoclonal
Application:
ELISA (i), WB
Citations:
3
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.
UniProt accession no.
shipped in
dry ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... CTNS(1497)
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
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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存储类别
10 - Combustible liquids
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
法规信息
新产品
此项目有
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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