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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.
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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.
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 (show LRMP Proteins), formed between two ATP-dependant gamma-glutamyl cycle enzymes, gamma-glutamyl cysteine synthetase and 5-oxoprolinase (show OPLAH Proteins)
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.
cystinosin-deficient cells had abnormal shape and distribution of the endo-lysosomal compartments and impaired endocytosis, with decreased surface expression of multiligand receptors and delayed lysosomal cargo processing.
CTNS-LKG represents 5-20 % of CTNS transcripts, with the exception of the testis that expresses both isoforms in equal proportions.
identified two novel CTNS splicing deletions in a Chinese IC family, one at the donor site of exon 6 of CTNS and the other at the acceptor site of exon 8
We recommend that black South African and Cape Coloured patients presenting with cystinosis be tested for CTNS-c.971-12G > A in the first instance, with the possibility of prenatal testing being offered to at-risk families.
Cystinosin, MPDU1, SWEETs and KDELR belong to a well-defined protein family with putative function of cargo receptors.[cytonosin]
results objectify the pigmentation defect in patients with cystinosis. We also identify the role of CTNS in melanogenesis and add a new gene to the list of the genes involved in the control of skin and hair pigmentation
Mutation analysis of CTNS in six cystinosis patients from four families in Thailand. Using PCR sequencing of the entire coding regions, study identified all eight mutant alleles, including two mutations, p.G309D and p.Q284X, that have not been previously reported.
Report CTNS mutations in Turkish cystinosis patients.
cystinosin exports the proteolysis-derived dimeric amino acid cystine from lysosomes and is impaired in cystinosis.
CTNS plays a pivotal role in regulating cell thiol concentrations.
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.
amino acid transport system xc-
, cysteine/glutamate transporter
, cystine/glutamate transporter
, sodium independent anionic amino acid transport system
, solute carrier family 7 member 11
, cystinosis, nephropathic
, cystinosin, lysosomal cystine transporter