Typical Testing Data/Standard Curve (for reference only)
Human Cystinosin (CTNS) ELISA Kit | CTNS elisa kit
Human Cystinosin (CTNS) ELISA Kit
Typical Testing Data/Standard Curve (for reference only)
Typical Testing Data/Standard Curve (for reference only)
Principle of the Assay: CTNS ELISA kit applies the competitive enzyme immunoassay technique utilizing a polyclonal anti-CTNS antibody and an CTNS-HRP conjugate. The assay sample and buffer are incubated together with CTNS-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the CTNS concentration since CTNS from samples and CTNS-HRP conjugate compete for the anti-CTNS antibody binding site. Since the number of sites is limited, as more sites are occupied by CTNS from the sample, fewer sites are left to bind CTNS-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The CTNS concentration in each sample is interpolated from this standard curve.
NCBI and Uniprot Product Information
Uniprot Description
CTNS: Thought to transport cystine out of lysosomes. Defects in CTNS are the cause of cystinosis nephropathic type (CTNS). It is a form of cystinosis, a lysosomal storage disease due to defective transport of cystine across the lysosomal membrane. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. The classical nephropathic form has onset in the first year of life and is characterized by a polyuro-polydipsic syndrome, marked height-weight growth delay, generalized impaired proximal tubular reabsorptive capacity, with severe fluid-electrolyte balance alterations, renal failure, ocular symptoms and other systemic complications. Defects in CTNS are the cause of cystinosis adult non- nephropathic type (CTNSANN). It is a form of cystinosis, a lysosomal storage disease due to defective transport of cystine across the lysosomal membrane. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. Cystinosis adult non-nephropathic type is characterized by ocular features and a benigne course. Patients manifest mild photophobia due to conjunctival and corneal cystine crystals. Defects in CTNS are the cause of cystinosis late-onset juvenile or adolescent nephropathic type (CTNSJAN). It is a form of cystinosis, a lysosomal storage disease due to defective transport of cystine across the lysosomal membrane. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. Late-onset juvenile or adolescent nephropathic cystinosis manifests itself first at age 10 to 12 years with proteinuria due to glomerular damage rather than with the manifestations of tubular damage that occur first in infantile cystinosis. There is no excess amino aciduria and stature is normal. Photophobia, late development of pigmentary retinopathy, and chronic headaches are features. Belongs to the cystinosin family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Membrane protein, multi-pass; Membrane protein, integral
Cellular Component: intermediate filament cytoskeleton; membrane; intracellular membrane-bound organelle; lysosomal membrane; lysosome; late endosome; integral to membrane; plasma membrane
Molecular Function: L-cystine transmembrane transporter activity
Biological Process: ATP metabolic process; melanin biosynthetic process; grooming behavior; lens development in camera-type eye; glutathione metabolic process; long-term memory; transport; L-cystine transport; visual learning; brain development; cognition; adult walking behavior
Research Articles on CTNS
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Product Notes
The Human CTNS ctns (Catalog #AAA7250849) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA7250849 ELISA Kit recognizes Human CTNS. It is sometimes possible for the material contained within the vial of "Cystinosin (CTNS), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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