Stefin B (CSTB) Mouse Monoclonal Antibody [Clone ID: M1-C1]
Other products for "CSTB"
Specifications
Product Data | |
Clone Name | M1-C1 |
Applications | ELISA, IHC, WB |
Recommended Dilution | ELISA. Immunohistochemistry on Paraffin Sections: 5 µg/ml. Western Blot. |
Reactivities | Human |
Host | Mouse |
Isotype | IgG1 |
Clonality | Monoclonal |
Immunogen | CSTB antibody was raised against cSTB (AAH03370.1, 1 a.a. ~ 98 a.a) full length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. |
Specificity | This antibody reacts to CSTB / Stefin B. |
Formulation | PBS, pH 7.2 State: Purified State: Liquid purified Ig fraction |
Concentration | lot specific |
Purification | Affinity chromatography on Protein A |
Storage | Store the antibody undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing. |
Stability | Shelf life: one year from despatch. |
Gene Name | Homo sapiens cystatin B (CSTB) |
Database Link | |
Background | 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) [MIM:254800]. 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. |
Synonyms | Stefin-B, CPI-B, CSTB, CST6, STFB |
Reference Data |
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