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Mouse Anti-ACTN1 Recombinant Antibody (CBT115) (V2LY-0625-LY3788)


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Summary

Host Animal
Mouse
Specificity
Human, Rat, Mouse
Clone
CBT115
Antibody Isotype
IgG
Application
IHC-P, IF

Basic Information

Immunogen
Recombinant Protein of a-actinin of ACTN1
Host Species
Mouse
Specificity
Human, Rat, Mouse
Antibody Isotype
IgG
Clonality
Monoclonal Antibody
Application Notes
ApplicationNote
IHC1:100-1:200

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

Format
Liquid
Buffer
PBS, Glycerol, BSA
Preservative
Sodium azide
Concentration
Batch dependent
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Actinin Alpha 1
Entrez Gene ID
87
UniProt ID
P12814
Alternative Names
Actinin Alpha 1; Alpha-Actinin Cytoskeletal Isoform; F-Actin Cross-Linking Protein; Non-Muscle Alpha-Actinin-1; Actinin 1 Smooth Muscle; Alpha-Actinin-1; BDPLT15;
Function
F-actin cross-linking protein which is thought to anchor actin to a variety of intracellular structures. This is a bundling protein.
Biological Process
Actin filament bundle assembly
Actin filament network formation
Actin filament organization
Focal adhesion assembly
Negative regulation of cellular component movement
Platelet degranulation
Platelet formation
Platelet morphogenesis
Regulation of apoptotic process
Cellular Location
Cytoskeleton; Cell membrane; Z line; Cell junction. Colocalizes with MYOZ2 and PPP3CA at the Z-line of heart and skeletal muscle. Colocalizes with PSD in membrane ruffles and central reticular structures.
Involvement in disease
Bleeding disorder, platelet-type 15 (BDPLT15): An autosomal dominant form of macrothrombocytopenia. Affected individuals usually have no or only mild bleeding tendency, such as epistaxis. Laboratory studies show decreased numbers of large platelets and anisocytosis, but the platelets show no in vitro functional abnormalities.
More Infomation

O'Sullivan, L. R., Ajaykumar, A. P., Dembicka, K. M., Murphy, A., Grennan, E. P., & Young, P. W. (2020). Investigation of calmodulin‐like and rod domain mutations suggests common molecular mechanism for α‐actinin‐1‐linked congenital macrothrombocytopenia. FEBS letters, 594(1), 161-174.

Xie, G. F., Zhao, L. D., Chen, Q., Tang, D. X., Chen, Q. Y., Lu, H. F., ... & Chen, Z. (2020). High ACTN1 is associated with poor prognosis, and ACTN1 silencing suppresses cell proliferation and metastasis in Oral squamous cell carcinoma. Drug Design, Development and Therapy, 14, 1717.

Cetinkaya, A., Berge, B., Sen-Hild, B., Troidl, K., Gajawada, P., Kubin, N., ... & Richter, M. (2020). Radixin relocalization and nonmuscle α-Actinin expression are features of remodeling cardiomyocytes in adult patients with dilated cardiomyopathy. Disease Markers, 2020.

Blondelle, J., Tallapaka, K., Seto, J. T., Ghassemian, M., Clark, M., Laitila, J. M., ... & Lange, S. (2019). Cullin-3–dependent deregulation of ACTN1 represents a pathogenic mechanism in nemaline myopathy. JCI insight, 4(10).

Eich, F., Nonis, D. F., Sen, N. E., Nowock, J., & Auburger, G. (2019). Ataxin-2 Binds Alpha-Actinin-1.

Yang, X., Pang, Y., Zhang, J., Shi, J., Zhang, X., Zhang, G., ... & Fu, L. (2019). High expression levels of ACTN1 and ACTN3 indicate unfavorable prognosis in acute myeloid leukemia. Journal of Cancer, 10(18), 4286.

Faleschini, M., Melazzini, F., Marconi, C., Giangregorio, T., Pippucci, T., Cigalini, E., ... & Noris, P. (2018). ACTN 1 mutations lead to a benign form of platelet macrocytosis not always associated with thrombocytopenia. British journal of haematology, 183(2), 276-288.

Kanhai, D., Mulder, R., Ploos van Amstel, H. K., Schutgens, R., Lukens, M., & Tamminga, R. Y. (2018). Familial macrothrombocytopenia due to a double mutation in cis in the alpha‐actinin 1 gene (ACTN1), previously considered to be chronic immune thrombocytopenic purpura. Pediatric blood & cancer, 65(12), e27418.

Milanini, J., Fayad, R., Partisani, M., Lecine, P., Borg, J. P., Franco, M., & Luton, F. (2018). EFA6 proteins regulate lumen formation through α-actinin 1. Journal of cell science, 131(3), jcs209361.

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

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