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

Name: Mouse Anti-OBSCN (AA 1551-1649) Recombinant Antibody (5C20)
SKU: CBMAB-O0234-CQ
Rating: 5 (1 reviews)

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Datasheet

SDS

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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

5C20

Application

WB, IP, ELISA

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

More Infomation

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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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
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For research use only. Not intended for any clinical use.

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