CTNS monoclonal antibody (M09), clone 5G6
* The price is valid only in USA. Please select country.
-
More Files
- More Functions
-
Specification
Product Description
Mouse monoclonal antibody raised against a partial recombinant CTNS.
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
Host
Mouse
Reactivity
Human, Mouse, Rat
Isotype
IgG2a Kappa
Quality Control Testing
Antibody Reactive Against Recombinant Protein.
Western Blot detection against Immunogen (36.74 KDa) .
Storage Buffer
In 1x PBS, pH 7.4
Storage Instruction
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
-
Applications
Western Blot (Tissue lysate)
CTNS monoclonal antibody (M09), clone 5G6. Western Blot analysis of CTNS expression in human liver.Western Blot (Cell lysate)
CTNS monoclonal antibody (M09), clone 5G6. Western Blot analysis of CTNS expression in Raw 264.7.Western Blot (Cell lysate)
CTNS monoclonal antibody (M09), clone 5G6. Western Blot analysis of CTNS expression in PC-12.Western Blot (Cell lysate)
CTNS monoclonal antibody (M09), clone 5G6. Western Blot analysis of CTNS expression in HepG2.Western Blot (Recombinant protein)
Sandwich ELISA (Recombinant protein)
Detection limit for recombinant GST tagged CTNS is approximately 0.3ng/ml as a capture antibody.ELISA
-
Gene Info — CTNS
Entrez GeneID
1497GeneBank Accession#
NM_004937Protein Accession#
NP_004928Gene Name
CTNS
Gene Alias
CTNS-LSB, PQLC4
Gene Description
cystinosis, nephropathic
Gene Ontology
HyperlinkGene Summary
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
Other Designations
-
-
Interactome
-
Pathway
-
Disease
-
Publication Reference
-
The aminoglycoside geneticin permits translational readthrough of the CTNS W138X nonsense mutation in fibroblasts from patients with nephropathic cystinosis.
Brasell EJ, Chu L, El Kares R, Seo JH, Loesch R, Iglesias DM, Goodyer P.
Pediatric Nephrology (Berlin, Germany) 2018 Nov; [Epub].
Application:WB-Ce, Human, Fibroblasts.
-
Cystine accumulation attenuates insulin release from the pancreatic beta-cell due to elevated oxidative stress and decreased ATP levels.
McEvoy B, Sumayao R, Slattery C, McMorrow T, Newsholme P.
The Journal of Physiology 2015 Dec; 593(23):5167.
Application:WB-Ce, Rat, BRIN-BD11 cells.
-
Modulation of CTNS Gene Expression By Intracellular Thiols.
Bellomo F, Corallini S, Pastore A, Palma A, Laurenzi C, Emma F, Taranta A.
Free Radical Biology & Medicine 2010 Apr; 48(7):865.
Application:WB-Tr, Human, HK-2 cells.
-
The aminoglycoside geneticin permits translational readthrough of the CTNS W138X nonsense mutation in fibroblasts from patients with nephropathic cystinosis.
- +1-909-264-1399
+1-909-992-0619
Toll Free : +1-877-853-6098 - +1-909-992-3401
- sales@abnova.com