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Human CSTB Protein expressed in Escherichia coli (E. coli) - ABIN666707
Järvinen, Rinne, Hopsu-Havu: Human cystatins in normal and diseased tissues--a review. in Acta histochemica 1988
Show all 2 references for ABIN666707
Human CSTB Protein expressed in Escherichia coli (E. coli) - ABIN2002843
Martin, Craven, Jerala, Kroon-Zitko, Zerovnik, Turk, Waltho: The three-dimensional solution structure of human stefin A. in Journal of molecular biology 1995
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 Proteins) in response to TLR4 (show TLR4 Proteins) 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 Proteins) 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 Proteins) 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 Proteins) in the nucleus
CSTB downregulation may promote the development of gastric cancer.
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 Proteins) and ANXA4 (show ANXA4 Proteins) 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 Proteins) 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 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