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Mouse Anti-OBSCN (AA 1551-1649) Recombinant Antibody (5C20) (CBMAB-O0234-CQ)

This product is a mouse antibody that recognizes OBSCN (AA 1551-1649). The antibody 5C20 can be used for immunoassay techniques such as: WB, IP, ELISA.
See all OBSCN antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
5C20
Application
WB, IP, ELISA

Basic Information

Immunogen
OBSCN (XP_290923, 1551aa-1649aa) partial Recombinant protein with GST tag
Specificity
Human
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
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
AA 1551-1649

Target

Full Name
obscurin, cytoskeletal calmodulin and titin-interacting RhoGEF
Introduction
The obscurin gene spans more than 150 kb, contains over 80 exons and encodes a protein of approximately 720 kDa. The encoded protein contains 68 Ig domains, 2 fibronectin domains, 1 calcium/calmodulin-binding domain, 1 RhoGEF domain with an associated PH domain, and 2 serine-threonine kinase domains. This protein belongs to the family of giant sacromeric signaling proteins that includes titin and nebulin, and may have a role in the organization of myofibrils during assembly and may mediate interactions between the sarcoplasmic reticulum and myofibrils. Alternatively spliced transcript variants encoding different isoforms have been identified.
Entrez Gene ID
84033
UniProt ID
Q5VST9
Alternative Names
Obscurin, Cytoskeletal Calmodulin And Titin-Interacting RhoGEF; Obscurin-MLCK; Obscurin, Myosin Light Chain Kinase; Obscurin-Myosin Light Chain Kinase; Obscurin-RhoGEF; EC 2.7.11.1
Function
Structural component of striated muscles which plays a role in myofibrillogenesis. Probably involved in the assembly of myosin into sarcomeric A bands in striated muscle (PubMed:11448995, PubMed:16205939).
Has serine/threonine protein kinase activity and phosphorylates N-cadherin CDH2 and sodium/potassium-transporting ATPase subunit ATP1B1 (By similarity).
Binds (via the PH domain) strongly to phosphatidylinositol 3,4-bisphosphate (PtdIns(3,4)P2) and phosphatidylinositol 4,5-bisphosphate (PtdIns(4,5)P2), and to a lesser extent to phosphatidylinositol 3-phosphate (PtdIns3P), phosphatidylinositol 4-phosphate (PtdIns4P), phosphatidylinositol 5-phosphate (PtdIns5P) and phosphatidylinositol 3,4,5-trisphosphate (PtdIns(3,4,5)P3) (PubMed:28826662).
Biological Process
Cell-cell adhesionManual Assertion Based On ExperimentIBA:GO_Central
Protein localization to M-bandBy SimilarityISS:BHF-UCL
Regulation of small GTPase mediated signal transductionTAS:Reactome
Sarcomere organizationManual Assertion Based On ExperimentTAS:BHF-UCL
Cellular Location
Isoform 3
Cytoplasm, myofibril, sarcomere, M line
Cytoplasm, myofibril, sarcomere, Z line
In differentiating skeletal muscle cells, isoform 3 primarily localizes to the sarcomeric M-line and less frequently to the Z-disk (PubMed:12527750).
Isoform 3 colocalizes with ANK1 isoform Mu17/ank1.5 at the M-line in differentiated skeletal muscle cells (PubMed:12527750).
Cytoplasm, myofibril, sarcomere, M line
Cytoplasm, myofibril, sarcomere, Z line
Cell membrane, sarcolemma
Nucleus
Colocalizes with CDH2 and ATP1B1 to the sarcolemma and to intercalating disks in cardiac muscles. Colocalizes with ATP1B1 to M line and Z line in cardiac muscles.
Involvement in disease
A chromosomal aberration involving OBSCN has been found in Wilms tumor. Translocation t(1;7)(q42;p15) with PTHB1.
PTM
Autophosphorylated by protein kinase domains 1 and 2.

Zemorshidi, F., Töpf, A., Claeys, K. G., McFarlane, A., Patton, A., Nafissi, S., & Straub, V. (2024). Novel OBSCN variants associated with a risk to exercise-intolerance and rhabdomyolysis. Neuromuscular Disorders, 34, 83-88.

Guardia, T., Zhang, Y., Thompson, K. N., Lee, S. J., Martin, S. S., Konstantopoulos, K., & Kontrogianni-Konstantopoulos, A. (2023). OBSCN restoration via OBSCN-AS1 long-noncoding RNA CRISPR-targeting suppresses metastasis in triple-negative breast cancer. Proceedings of the National Academy of Sciences, 120(11), e2215553120.

Da, M., Xu, J., Ma, S., Yang, Z., Xu, Y., Qi, J., & Mo, X. (2023). Novel compound heterozygous OBSCN variants in Chinese children with congenital pulmonary airway malformation.

Cabrera-Serrano, M., Caccavelli, L., Savarese, M., Vihola, A., Jokela, M., Johari, M., ... & Ravenscroft, G. (2022). Bi-allelic loss-of-function OBSCN variants predispose individuals to severe recurrent rhabdomyolysis. Brain, 145(11), 3985-3998.

Gao, E., Liu, X., Yang, Y., He, Y., & Xue, D. (2022). OBSCN as biomarker for evaluating tumor mutation burden and anti-tumor immunity in Stomach adenocarcinoma.

Liu, Z., Wang, L., Guo, C., Liu, L., Jiao, D., Sun, Z., ... & Han, X. (2021). TTN/OBSCN ‘Double‐Hit’predicts favourable prognosis,‘immune‐hot’subtype and potentially better immunotherapeutic efficacy in colorectal cancer. Journal of Cellular and Molecular Medicine, 25(7), 3239-3251.

Wu, G., Liu, J., Liu, M., Huang, Q., Ruan, J., Zhang, C., ... & Song, L. (2021). Truncating variants in OBSCN gene associated with disease-onset and outcomes of hypertrophic cardiomyopathy. Circulation: Genomic and Precision Medicine, 14(5), e003401.

Chen, P., Xiao, Y., Wang, Y., Zheng, Z., Chen, L., Yang, X., ... & Zhang, S. (2020). Intracellular calcium current disorder and disease phenotype in OBSCN mutant iPSC-based cardiomyocytes in arrhythmogenic right ventricular cardiomyopathy. Theranostics, 10(24), 11215.

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

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