anti-Cystinosis, Nephropathic (CTNS) Anticorps

CTNS encodes a seven-transmembrane domain protein that functions to transport cystine out of lysosomes. De plus, nous expédions CTNS Protéines (9) et CTNS Kits (5) et beaucoup plus de produits pour cette protéine.

afficher tous les anticorps Gène GeneID UniProt
CTNS 1497 O60931
CTNS 287478  
CTNS 83429 P57757
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Top anti-CTNS Anticorps sur anticorps-enligne.fr

Showing 10 out of 47 products:

Catalogue No. Reactivité Hôte Conjugué Application Images Quantité Fournisseur Livraison Prix Détails
Boeuf (Vache) Lapin Inconjugué WB Host: Rabbit Target Name: Ctns Sample Type: Mouse Lung lysates Antibody Dilution: 1.0ug/ml 100 μL Connectez-vous pour afficher 2 to 3 Days
$289.00
Détails
Humain Souris Inconjugué ELISA, WB CTNS monoclonal antibody (M09), clone 5G6 Western Blot analysis of CTNS expression in HepG2. Detection limit for recombinant GST tagged CTNS is approximately 0.3 ng/mL as a capture antibody. 100 μg Connectez-vous pour afficher 8 to 11 Days
$461.00
Détails
Humain Souris Inconjugué ELISA, WB CTNS monoclonal antibody (M09), clone 5G6. Western Blot analysis of CTNS expression in HepG2. Detection limit for recombinant GST tagged CTNS is approximately 0.3ng/ml as a capture antibody. 100 μg Connectez-vous pour afficher 11 to 12 Days
$440.00
Détails
Humain Lapin Inconjugué WB CTNS Antibody (C-term) (ABIN1881240) western blot analysis in HepG2 cell line lysates (35 µg/lane). This demonstrates the CTNS antibody detected the CTNS protein (arrow). 400 μL Connectez-vous pour afficher 10 to 11 Days
$335.50
Détails
Humain Lapin Inconjugué IHC (p), IHC, ELISA Immunohistochemistry-Paraffin: CTNS Antibody [NBP1-02469] - Staining of human skeletal muscle. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 25 ug/ml. 0.05 mg Connectez-vous pour afficher 10 to 13 Days
$556.31
Détails
Humain Lapin Inconjugué EIA, IHC (p) 50 μg Connectez-vous pour afficher 6 to 8 Days
$390.50
Détails
Humain Lapin Inconjugué IF/ICC, WB Western blot analysis of extracts of various cell lines, using CTNS antibody (ABIN5974151) at 1/1000 dilution. Immunofluorescence analysis of HeLa cells using CTNS antibody (ABIN5974151). Blue: DAPI for nuclear staining. 100 μL Connectez-vous pour afficher 11 to 16 Days
$426.40
Détails
Humain Lapin Inconjugué IF, WB Western blot analysis of extracts of various cell lines, using CTNS antibody. Immunofluorescence analysis of HeLa cells using CTNS antibody. 120 μL Connectez-vous pour afficher 12 to 14 Days
$251.90
Détails
Humain Lapin Inconjugué WB Western Blot analysis of CTNS expression in transfected 293T cell line by CTNS MaxPab polyclonal antibody.<br><br>Lane 1: CTNS transfected lysate(45.10 KDa).<br>Lane 2: Non-transfected lysate.<br> CTNS MaxPab rabbit polyclonal antibody. Western Blot analysis of CTNS expression in human stomach. 100 μg Connectez-vous pour afficher 11 to 12 Days
$425.71
Détails
Humain Lapin Inconjugué IF, IHC, WB Western blot analysis of extracts of various cell lines, using CTNS antibody. Immunofluorescence analysis of HeLa cell using CTNS antibody. Blue: DAPI for nuclear staining. 100 μL Connectez-vous pour afficher 16 Days
$334.02
Détails

anti-CTNS Anticorps mieux référencés

  1. 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 (PubMed)
    Show all 5 Pubmed References

  2. 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 (PubMed)
    Show all 2 Pubmed References

  3. 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 (PubMed)

Plus d’anticorps contre CTNS partenaires d’interaction

Human Cystinosis, Nephropathic (CTNS) interaction partners

  1. Cystinosis was primarily diagnosed by a pediatric nephrologist and then referred to the Iran University of Medical Sciences genetics clinic for consultation and molecular analysis, which involved polymerase chain reaction (PCR) amplification to determine the presence or absence of the 57-kb founder deletion in CTNS, followed by direct sequencing of the coding exons of CTNS

  2. Potential dual function of PQ-loop proteins such as cystinosin.

  3. 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.

  4. 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.

  5. 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.

  6. 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.

  7. silencing of AP-2 (Montrer GTF3A Anticorps) triggers the clathrin-independent endocytosis, showing the complex adaptability of cystinosin-LKG trafficking

  8. 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.

  9. GCK (Montrer GCK Anticorps) mutations are associated with Cystinosis.

  10. Lack of cystinosin reduced TFEB (Montrer TFEB Anticorps) expression and induced TFEB (Montrer TFEB Anticorps) nuclear translocation.

Mouse (Murine) Cystinosis, Nephropathic (CTNS) interaction partners

  1. 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.

  2. 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.

  3. Cells and tissues lacking CTNS expression are characterized by increased autophagosome numbers, but functional macroautophagic flux.

  4. The Ctns(-/-) mouse model generated on C57BL/6 background is not suitable for clarifying the pathogenesis of male infertility in cystinosis.

  5. Deletion of cystinosis gene (Ctns) causes corneal cystine crystals formation, neovascularization and scarring in the cornea of ctns transgenic mice mimicking cystinosis in humans.

  6. 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)

  7. CTNS has a role in proper functioning of the retina and bones, and in mouse behavior

  8. 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.

CTNS profil antigène

Profil protéine

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.

Gene names and symbols associated with CTNS

  • cystinosin, lysosomal cystine transporter (CTNS) anticorps
  • cystinosin, lysosomal cystine transporter (Ctns) anticorps
  • cystinosis, nephropathic (Ctns) anticorps
  • cystinosin, lysosomal cystine transporter (ctns) anticorps
  • cystinosis, nephropathic (CTNS) anticorps
  • cystinosin, lysosomal cystine transporter L homeolog (ctns.L) anticorps
  • AI195360 anticorps
  • AW049661 anticorps
  • CTNS anticorps
  • CTNS-LSB anticorps
  • PQLC4 anticorps
  • zgc:110194 anticorps

Protein level used designations for CTNS

cystinosin , cystinosis, nephropathic , cystinosin-like , cystinosin, lysosomal cystine transporter

GENE ID SPECIES
1497 Homo sapiens
417606 Gallus gallus
287478 Rattus norvegicus
83429 Mus musculus
553594 Danio rerio
491220 Canis lupus familiaris
100060554 Equus caballus
748043 Pan troglodytes
706721 Macaca mulatta
780074 Xenopus (Silurana) tropicalis
613527 Bos taurus
431937 Xenopus laevis
100223329 Taeniopygia guttata
100409361 Callithrix jacchus
100443212 Pongo abelii
100592953 Nomascus leucogenys
Fournisseurs de qualité sélectionnés pour anti-CTNS (CTNS) Anticorps
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