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Mouse Anti-CSTB Recombinant Antibody (CBXC-0599) (CBMAB-C3945-CQ)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXC-0599
Antibody Isotype
IgG1, κ
Application
ELISA, IF, IHC-P, WB

Basic Information

Immunogen
CSTB (AAH03370.1, 1 a.a.-98 a.a) full length recombinant protein with GST tag.
Specificity
Human
Antibody Isotype
IgG1, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.2
Concentration
0.5 mg/mL
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Cystatin B
Introduction
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.
Entrez Gene ID
1476
UniProt ID
P04080
Alternative Names
Cystatin B; Liver Thiol Proteinase Inhibitor; Cystatin B (Stefin B); CPI-B; STFB; CST6; 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
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.
More Infomation

Daura, E., Tegelberg, S., Yoshihara, M., Jackson, C., Simonetti, F., Aksentjeff, K., ... & Joensuu, T. (2021). Cystatin B-deficiency triggers ectopic histone H3 tail cleavage during neurogenesis. Neurobiology of Disease, 156, 105418.

Wu, Y., Whittaker, H. T., Noy, S., Cleverley, K., Brault, V., Herault, Y., ... & Wiseman, F. K. (2021). The effects of Cstb duplication on APP/amyloid-β pathology and cathepsin B activity in a mouse model. Plos one, 16(7), e0242236.

Di Matteo, F., Pipicelli, F., Kyrousi, C., Tovecci, I., Penna, E., Crispino, M., ... & Cappello, S. (2020). Cystatin B is essential for proliferation and interneuron migration in individuals with EPM 1 epilepsy. EMBO molecular medicine, 12(6), e11419.

Guan, W., Wang, X., Lin, Q., Zhang, J., Ren, W., & Xu, G. (2019). Transforming growth factor‑β/miR‑143‑3p/cystatin B axis is a therapeutic target in human ovarian cancer. International journal of oncology, 55(1), 267-276.

Penna, E., Cerciello, A., Chambery, A., Russo, R., Cernilogar, F. M., Pedone, E. M., ... & Crispino, M. (2019). Cystatin B involvement in synapse physiology of rodent brains and human cerebral organoids. Frontiers in Molecular Neuroscience, 195.

Unić, A., Derek, L., Duvnjak, M., Patrlj, L., Rakić, M., Kujundžić, M., ... & Grgurević, I. (2018). Diagnostic specificity and sensitivity of PIVKAII, GP3, CSTB, SCCA1 and HGF for the diagnosis of hepatocellular carcinoma in patients with alcoholic liver cirrhosis. Annals of Clinical Biochemistry, 55(3), 355-362.

O'Brien, A., Marshall, C. R., Blaser, S., Ray, P. N., & Yoon, G. (2017). Severe neurodegeneration, progressive cerebral volume loss and diffuse hypomyelination associated with a homozygous frameshift mutation in CSTB. European Journal of Human Genetics, 25(6), 775-778.

Ma, Y., Chen, Y., & Petersen, I. (2017). Expression and epigenetic regulation of cystatin B in lung cancer and colorectal cancer. Pathology-Research and Practice, 213(12), 1568-1574.

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For research use only. Not intended for any clinical use.

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We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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