Testing Data
Rabbit anti-Human Cystatin-B Polyclonal Antibody | anti-CSTB antibody
Rabbit anti-human Cystatin-B polyclonal Antibody, HRP conjugated
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Testing Data
Testing Data
NCBI and Uniprot Product Information
NCBI Description
The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins and kininogens. This gene encodes a stefin that functions as an intracellular thiol protease inhibitor. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. The protein is thought to play a role in protecting against the proteases leaking from lysosomes. Evidence indicates that mutations in this gene are responsible for the primary defects in patients with progressive myoclonic epilepsy (EPM1). [provided by RefSeq, Jul 2008]
Uniprot Description
CSTB: This is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B. Defects in CSTB are the cause of progressive myoclonic epilepsy type 1 (EPM1). EPM1 is an autosomal recessive disorder characterized by severe, stimulus-sensitive myoclonus and tonic-clonic seizures. The onset, occurring between 6 and 13 years of age, is characterized by convulsions. Myoclonus begins 1 to 5 years later. The twitchings occur predominantly in the proximal muscles of the extremities and are bilaterally symmetrical, although asynchronous. At first small, they become late in the clinical course so violent that the victim is thrown to the floor. Mental deterioration and eventually dementia develop. Belongs to the cystatin family.
Protein type: Inhibitor
Chromosomal Location of Human Ortholog: 21q22.3
Cellular Component: cytoplasm; extracellular space; nucleolus
Molecular Function: cysteine protease inhibitor activity; endopeptidase inhibitor activity; protease binding
Biological Process: adult locomotory behavior; negative regulation of peptidase activity; negative regulation of proteolysis
Disease: Myoclonic Epilepsy Of Unverricht And Lundborg
Research Articles on CSTB
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Product Notes
The CSTB cstb (Catalog #AAA715547) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Rabbit anti-human Cystatin-B polyclonal Antibody, HRP conjugated reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Cystatin-B can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA). Researchers should empirically determine the suitability of the CSTB cstb for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Cystatin-B, Polyclonal Antibody" 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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