CSTB
CSTB (Cystatin B) is a Protein Coding gene. Diseases associated with CSTB include Myoclonic Epilepsy Of Unverricht And Lundborg and Unverricht-Lundborg Syndrome. Among its related pathways are Innate Immune System. Gene Ontology (GO) annotations related to this gene include endopeptidase inhibitor activity. An important paralog of this gene is CSTA.
Full Name
Cystatin B
Function
This is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B.
Biological Process
Adult locomotory behavior Source: Ensembl
Negative regulation of peptidase activity Source: BHF-UCL
Negative regulation of proteolysis Source: UniProtKB
Neutrophil degranulation Source: Reactome
Negative regulation of peptidase activity Source: BHF-UCL
Negative regulation of proteolysis Source: UniProtKB
Neutrophil degranulation Source: Reactome
Cellular Location
Cytoplasm; Nucleus
Involvement in disease
Epilepsy, progressive myoclonic 1 (EPM1):
A form of progressive myoclonic epilepsy, a clinically and genetically heterogeneous group of disorders defined by the combination of action and reflex myoclonus, other types of epileptic seizures, and progressive neurodegeneration and neurocognitive impairment. EPM1 is an autosomal recessive form 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.
A form of progressive myoclonic epilepsy, a clinically and genetically heterogeneous group of disorders defined by the combination of action and reflex myoclonus, other types of epileptic seizures, and progressive neurodegeneration and neurocognitive impairment. EPM1 is an autosomal recessive form 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.
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Anti-CSTB antibodies
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CompareTarget: CSTB
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBXS-2395
Application*: WB
Target: CSTB
Host: Mouse
Antibody Isotype: IgG2b, λ
Specificity: Mouse, Rat
Clone: CBXC-0598
Application*: E, P, WB, IF
Target: CSTB
Host: Rat
Antibody Isotype: IgG2a
Specificity: Mouse
Clone: CBFYC-2360
Application*: WB, IP
Target: CSTB
Host: Rat
Antibody Isotype: IgG2
Specificity: Mouse
Clone: CBFYC-2357
Application*: IH, WB
Target: CSTB
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: M2-F1
Application*: E, IF, IH, WB
Target: CSTB
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: S6498
Application*: P, WB
Target: CSTB
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human, Rat
Clone: CBXS-1502
Application*: WB, F, P
Target: CSTB
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBXS-5510
Application*: F, IF, IH, WB
Target: CSTB
Host: Mouse
Antibody Isotype: IgG2
Specificity: Human
Clone: CBXS-5502
Application*: N, WB
Target: CSTB
Host: Mouse
Antibody Isotype: IgG2b, κ
Specificity: Mouse, Rat
Clone: CBXS-4564
Application*: IF, IH, WB
Target: CSTB
Host: Mouse
Antibody Isotype: IgG2b, κ
Specificity: Mouse, Rat
Clone: CBXC-0599
Application*: IF, IH, WB
Target: CSTB
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CBXC-1024
Application*: WB, IP, N
Target: CSTB
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBXC-0600
Application*: E, IP, WB
Target: CSTB
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: M1-C1
Application*: E, WB, IH
Target: CSTB
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBXC-2106
Application*: WB, IP, IF
Target: CSTB
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBFYC-2363
Application*: WB, IP, IF
Target: CSTB
Host: Rat
Antibody Isotype: IgG2a
Specificity: Mouse
Clone: CBFYC-2361
Application*: IH
Target: CSTB
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human, Mouse, Rat
Clone: CBFYC-2359
Application*: WB, IP, IF, E, P
Target: CSTB
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBFYC-2358
Application*: E
Target: CSTB
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: 06
Application*: E
Target: CSTB
Specificity: Human
Target: CSTB
Specificity: Human
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(P): Predicted
* Abbreviations
- AActivation
- AGAgonist
- APApoptosis
- BBlocking
- BABioassay
- BIBioimaging
- CImmunohistochemistry-Frozen Sections
- CIChromatin Immunoprecipitation
- CTCytotoxicity
- CSCostimulation
- DDepletion
- DBDot Blot
- EELISA
- ECELISA(Cap)
- EDELISA(Det)
- ESELISpot
- EMElectron Microscopy
- FFlow Cytometry
- FNFunction Assay
- GSGel Supershift
- IInhibition
- IAEnzyme Immunoassay
- ICImmunocytochemistry
- IDImmunodiffusion
- IEImmunoelectrophoresis
- IFImmunofluorescence
- IGImmunochromatography
- IHImmunohistochemistry
- IMImmunomicroscopy
- IOImmunoassay
- IPImmunoprecipitation
- ISIntracellular Staining for Flow Cytometry
- LALuminex Assay
- LFLateral Flow Immunoassay
- MMicroarray
- MCMass Cytometry/CyTOF
- MDMeDIP
- MSElectrophoretic Mobility Shift Assay
- NNeutralization
- PImmunohistologyp-Paraffin Sections
- PAPeptide Array
- PEPeptide ELISA
- PLProximity Ligation Assay
- RRadioimmunoassay
- SStimulation
- SESandwich ELISA
- SHIn situ hybridization
- TCTissue Culture
- WBWestern Blot
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