Cystinosis, Nephropathic Proteins (CTNS)

CTNS encodes a seven-transmembrane domain protein that functions to transport cystine out of lysosomes. Additionally we are shipping CTNS Antibodies (45) and CTNS Kits (3) and many more products for this protein.

list all proteins Gene Name GeneID UniProt
CTNS 1497 O60931
Rat CTNS CTNS 287478  
CTNS 83429 P57757
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Top CTNS Proteins at antibodies-online.com

Showing 7 out of 8 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
Escherichia coli (E. coli) Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 30 to 35 Days
$4,744.22
Details
Insect Cells Human rho-1D4 tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 0.5 mg Log in to see 50 to 55 Days
$6,616.87
Details
Insect Cells Mouse rho-1D4 tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 0.25 mg Log in to see 50 to 55 Days
$4,649.05
Details
Escherichia coli (E. coli) Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 30 to 35 Days
$4,744.22
Details
Wheat germ Human GST tag 10 μg Log in to see 11 to 12 Days
$414.29
Details
HEK-293 Cells Human Myc-DYKDDDDK Tag Validation with Western Blot 20 μg Log in to see 10 to 12 Days
$860.20
Details
Escherichia coli (E. coli) Human His tag   100 μg Log in to see 15 to 18 Days
$720.00
Details

CTNS Proteins by Origin and Source

Origin Expressed in Conjugate
Human , , ,
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Mouse (Murine) ,
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More Proteins for Cystinosis, Nephropathic (CTNS) Interaction Partners

Human Cystinosis, Nephropathic (CTNS) interaction partners

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

  2. 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 (show TNF Proteins)- and actinomycin D-inducted apoptosis dropped in cystinotic cells expressing LKG-isoform. This effect was also similar to the main isoform.

  3. This work demonstrated no major abnormality ofER and lysosomal Ca2 (show CA2 Proteins)+signalling associated with cystinosin defi-ciency in human proximal tubular epithelial cells.

  4. 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.

  5. 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.

  6. silencing of AP-2 (show GTF3A Proteins) triggers the clathrin-independent endocytosis, showing the complex adaptability of cystinosin-LKG trafficking

  7. 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.

  8. GCK (show GCK Proteins) mutations are associated with Cystinosis.

  9. Lack of cystinosin reduced TFEB (show TFEB Proteins) expression and induced TFEB (show TFEB Proteins) nuclear translocation.

  10. CTNS deficiency alters cell signaling cascades resulting in impaired cell adhesion and enhanced cell motility in cystinosis.

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 (show FUT1 Proteins)) 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 (show LRMP Proteins), formed between two ATP-dependant gamma-glutamyl cycle enzymes, gamma-glutamyl cysteine synthetase and 5-oxoprolinase (show OPLAH Proteins)

  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 Protein Profile

Protein 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.

Gene names and symbols associated with CTNS

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

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
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