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The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Additionally we are shipping Cystatin B (Stefin B) Antibodies (224) and Cystatin B (Stefin B) Proteins (34) and many more products for this protein.
Showing 6 out of 21 products:
Human CSTB ELISA Kit for Sandwich ELISA - ABIN1889374
Alakurtti, Weber, Rinne, Theil, de Haan, Lindhout, Salmikangas, Saukko, Lahtinen, Lehesjoki: Loss of lysosomal association of cystatin B proteins representing progressive myoclonus epilepsy, EPM1, mutations. in European journal of human genetics : EJHG 2005
The interaction between AtCYSb and AtCaN2 AtCYSb regulates nucleic acid degradation in cells
Data suggests that AtCYS6 expression is enhanced by the germination inhibitory phytohormone ABA and that it participates in the control of germination rate and seedling growth by inhibiting the activity of stored cysteine proteinases. [AtCYS6]
Deletion of the encoding Cstb gene in Npc1-deficient mice resulted in striking deleterious effects, particularly within the cerebellum where diffuse loss of Purkinje cells was observed in young mice. This may be a consequence of damage to lysosomal membranes by reactive oxygen species (ROS), leading to the leakage of lysosomal contents that culminates in apoptosis.
Results suggest a role for CSTB in regulating microglial activation: data link CSTB deficiency to early activation and altered functional properties of microglia, imply presence of both enhanced and suppressed immune response-related microglial functions
the LPS-induced sepsis in StB KO mice is dependent on caspase-11 and mitochondrial reactive oxygen species but is not associated with the lysosomal destabilization and increased cathepsin activity in the cytosol
These results indicate an in vivo role for Stfb in protecting cancer cells by promoting their resistance to oxidative stress and to apoptosis induced through the lysosomal pathway.
Sefin B influences the expression of anti-inflammatory IL-10 (show IL10 ELISA Kits) in response to TLR4 (show TLR4 ELISA Kits) agonists.
An increase in Cstb does not induce any spontaneous epileptic activity.
Pathologic events in the CSTB-deficient brain highlight the potential role of glial activation at the initial stages of progressive myoclonic epilepsy type 1.
this study suggesting that the Cstb-Prmt2 (show PRMT2 ELISA Kits) region is not playing a major role in locomotor and cognitive deficits found in mice model of Down syndrome.
Data show that cystatin C (show CST3 ELISA Kits) effectively rescues cystatin B loss-of-function mutation, facilitating the reversal of pathophysiological changes and suggesting a novel therapeutic intervention for patients with neurodegenerative disorders.
Stefin B interacts with histones and cathepsin L (show CTSL1 ELISA Kits) in the nucleus
It was shown that decreased expression of cystatin B enhances cathepsin activity in Niemann-Pick C cerebellar degeneration patient fibroblasts.
High expression of stefin B may be an important factor contributing to the development and metastasis of Hepatocellular Carcinoma.
CSTB null mutation associated with microcephaly, early developmental delay, and severe dyskinesia.
Data shows that CYTB (show MT-CYB ELISA Kits) and ANXA4 (show ANXA4 ELISA Kits) overexpression may be involved in carcinogenesis and histopathological differentiation of ovarian clear cell carcinoma and suggest they may serve as a potential diagnostic biomarkers.
A role for disease-causing mutations in cystatin B gene in patients with juvenile myoclonic epilepsy was not supported.
Even though the majority of EPM1 patients have a uniform genetic mutation, the actual size of the longer CSTB expansion mutation allele is likely to have a modulating effect on the age at disease onset, myoclonus severity, and cortical neurophysiology.
The study shows detection of stefin B dimers in HEK293 cells and the importance of their residual activity.
glutamate (show GRIN1 ELISA Kits) dehydrogenase is a euchromatin-associated enzyme, and its H3 clipping activity is regulated by chromatin structure, histone modifications and an in vivo inhibitor.
detected a homozygous expansion of dodecamer repeats in the CSTB gene in four patients with clinical diagnosis of ULD.
The increased CSTB expression in ovarian tissue represents tumor progression and is dysregulated by the TGF-beta (show TGFB1 ELISA Kits) signaling pathway.
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).
cystatin B (stefin B)
, stefin B
, cystatin B
, Leukocyte cysteine proteinase inhibitor 1
, liver thiol proteinase inhibitor
, cystatin beta
, cystatin B protein
, stefin C