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CTNS encodes a seven-transmembrane domain protein that functions to transport cystine out of lysosomes. De plus, nous expédions CTNS Protéines (8) et CTNS Kits (3) et beaucoup plus de produits pour cette protéine.
Showing 10 out of 45 products:
Human Polyclonal CTNS Primary Antibody pour WB - ABIN1881240
Taranta, Wilmer, van den Heuvel, Bencivenga, Bellomo, Levtchenko, Emma: Analysis of CTNS gene transcripts in nephropathic cystinosis. dans Pediatric nephrology (Berlin, Germany) 2010
Show all 5 Pubmed References
Human Monoclonal CTNS Primary Antibody pour ELISA, WB - ABIN560527
Bellomo, Corallini, Pastore, Palma, Laurenzi, Emma, Taranta: Modulation of CTNS gene expression by intracellular thiols. dans Free radical biology & medicine 2010
Show all 2 Pubmed References
Human Polyclonal CTNS Primary Antibody pour WB - ABIN514757
Sumayao, McEvoy, Martin-Martin, McMorrow, Newsholme: Cystine dimethylester loading promotes oxidative stress and a reduction in ATP independent of lysosomal cystine accumulation in a human proximal tubular epithelial cell line. dans Experimental physiology 2013
Potential dual function of PQ-loop proteins such as cystinosin.
To study the role of the cystinosin-LKG-isoform, we have investigated cystine accumulation and apoptosis that have been described in cystinotic cells. The levels of TNFalpha (Montrer TNF Anticorps)- and actinomycin D-inducted apoptosis dropped in cystinotic cells expressing LKG-isoform. This effect was also similar to the main isoform.
This work demonstrated no major abnormality ofER and lysosomal Ca2 (Montrer CA2 Anticorps)+signalling associated with cystinosin defi-ciency in human proximal tubular epithelial cells.
The coding exons of the CTNS gene in 5 different Jordanian families and one family from Sudan with nephropathic cystinosis were sequenced. None had the European 57-kb deletion. 7 variants in the coding and promoter sequence of the CTNS gene were found: 294C>T, -180T>C, -118C>T, c.504G>A, p.Thr168Thr, c.829dupA in exon 10, and c.890G>A in exon 11.
Results show that the high turnover of ITILELP mutation (del AA67-73) in cystinosin, because of its immature glycosylation state together with low transport activity, might be responsible for the phenotype observed in some cystinosis patients who carry this mutation.
silencing of AP-2 (Montrer GTF3A Anticorps) triggers the clathrin-independent endocytosis, showing the complex adaptability of cystinosin-LKG trafficking
upon comparison of the patients with cystinosis in this particular region with the European and North American patients, it is clear that different CTNS variants result in this disease.
GCK (Montrer GCK Anticorps) mutations are associated with Cystinosis.
Lack of cystinosin reduced TFEB (Montrer TFEB Anticorps) expression and induced TFEB (Montrer TFEB Anticorps) nuclear translocation.
CTNS deficiency alters cell signaling cascades resulting in impaired cell adhesion and enhanced cell motility in cystinosis.
LAMP2A trafficking is regulated by cystinosin, Rab11, and RILP and that CMA up-regulation is a potential clinically relevant mechanism to increase cell survival in cystinosis.
Data indicate that hematopoietic stem cell (HSC (Montrer FUT1 Anticorps)) transplantation in cystinosin knockout (Ctns-/-) thyroid drastically decreased cystine accumulation and normalized the thyroid-stimulating hormone level.
Cells and tissues lacking CTNS expression are characterized by increased autophagosome numbers, but functional macroautophagic flux.
The Ctns(-/-) mouse model generated on C57BL/6 background is not suitable for clarifying the pathogenesis of male infertility in cystinosis.
Deletion of cystinosis gene (Ctns) causes corneal cystine crystals formation, neovascularization and scarring in the cornea of ctns transgenic mice mimicking cystinosis in humans.
the cause of cellular ATP depletion in nephrotic cystinosis may be the futile cycle (Montrer LRMP Anticorps), formed between two ATP-dependant gamma-glutamyl cycle enzymes, gamma-glutamyl cysteine synthetase and 5-oxoprolinase (Montrer OPLAH Anticorps)
CTNS has a role in proper functioning of the retina and bones, and in mouse behavior
Temporospatial pattern of cystine accumulation in Ctns-/- mice parallels that of patients and validates the mice as a model for the ocular anomalies of cystinosis.
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.
, cystinosis, nephropathic
, cystinosin, lysosomal cystine transporter