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Rat Anti-CSTB Recombinant Antibody (CBFYC-2360) (CBMAB-C2432-FY)


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Summary

Host Animal
Rat
Specificity
Mouse
Clone
CBFYC-2360
Antibody Isotype
IgG2a
Application
WB, IP

Basic Information

Immunogen
Recombinant mouse Cystatin B, Met2-Phe98.
Host Species
Rat
Specificity
Mouse
Antibody Isotype
IgG2a
Clonality
Monoclonal Antibody
Application Notes
ApplicationNote
WB1 μg/mL
IP25 μg/mL

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

Format
Lyophilized
Buffer
PBS, Trehalose
Preservative
None

Target

Entrez Gene ID
13014
UniProt ID
Q62426
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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