CSTB Antibody
Description
Product Specs
Product Science Overview
Cystatin B, also known as Stefin B, is a protein encoded by the CSTB gene in humans. It belongs to the cystatin superfamily, which comprises proteins that function as cysteine protease inhibitors. These inhibitors play a crucial role in regulating proteolytic activities within cells, thereby maintaining cellular homeostasis .
Cystatin B is an intracellular thiol proteinase inhibitor that tightly binds and reversibly inhibits cathepsins L, H, and B . These cathepsins are lysosomal proteases involved in various cellular processes, including protein degradation, antigen presentation, and apoptosis. By inhibiting these proteases, Cystatin B helps prevent excessive proteolysis, which can lead to cellular damage and disease.
Mutations in the CSTB gene are associated with a rare genetic disorder known as progressive myoclonic epilepsy type 1A (EPM1A), also referred to as Unverricht-Lundborg disease . This condition is characterized by myoclonic seizures, ataxia, and cognitive decline. The exact mechanism by which CSTB mutations lead to EPM1A is not fully understood, but it is believed that the loss of Cystatin B’s inhibitory function results in increased protease activity, leading to neuronal damage and neurodegeneration.
Mouse anti-human Cystatin B antibodies are monoclonal antibodies specifically designed to target and bind to human Cystatin B. These antibodies are commonly used in various research applications, including immunohistochemistry (IHC), immunocytochemistry (ICC), Western blotting (WB), and flow cytometry . They are valuable tools for studying the expression, localization, and function of Cystatin B in different biological contexts.
- Immunohistochemistry (IHC): Mouse anti-human Cystatin B antibodies are used to detect Cystatin B in tissue sections. This technique helps researchers study the distribution and localization of Cystatin B in various tissues and understand its role in different physiological and pathological conditions .
- Immunocytochemistry (ICC): These antibodies are also used to visualize Cystatin B in cultured cells. ICC allows researchers to examine the subcellular localization of Cystatin B and investigate its interactions with other cellular components .
- Western Blotting (WB): In WB, mouse anti-human Cystatin B antibodies are used to detect Cystatin B in protein extracts from cells or tissues. This technique helps quantify Cystatin B levels and assess its expression under different experimental conditions .
- Flow Cytometry: Flow cytometry with mouse anti-human Cystatin B antibodies enables the analysis of Cystatin B expression in individual cells within a mixed population. This application is particularly useful for studying cell-specific expression patterns and identifying cell types that express Cystatin B .
