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Mouse Anti-LMOD1 Recombinant Antibody (CBYCL-405) (CBMAB-L0306-YC)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBYCL-405
Antibody Isotype
IgG2a, κ
Application
ELISA, WB

Basic Information

Immunogen
Recombiant full length LMOD1 protein
Specificity
Human
Antibody Isotype
IgG2a, 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!]

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 1-270

Target

Introduction
The leiomodin 1 protein has a putative membrane-spanning region and 2 types of tandemly repeated blocks. The transcript is expressed in all tissues tested, with the highest levels in thyroid, eye muscle, skeletal muscle, and ovary. Increased expression of leiomodin 1 may be linked to Graves' disease and thyroid-associated ophthalmopathy.
Entrez Gene ID
25802
UniProt ID
P29536
Alternative Names
SMLMOD; 64kD; 1D; SM-LMOD; D1
Function
Required for proper contractility of visceral smooth muscle cells (PubMed:28292896).
Mediates nucleation of actin filaments.
Biological Process
Actin filament organizationManual Assertion Based On ExperimentIBA:GO_Central
Actin nucleationManual Assertion Based On ExperimentIDA:UniProtKB
Muscle contractionManual Assertion Based On ExperimentIBA:GO_Central
Myofibril assemblyManual Assertion Based On ExperimentIBA:GO_Central
Pointed-end actin filament cappingIEA:InterPro
Positive regulation of actin filament polymerizationManual Assertion Based On ExperimentIDA:UniProtKB
Cellular Location
Cytoplasm, myofibril, sarcomere
Cytoplasm, cytoskeleton
Colocalizes with actin filaments in sarcomeres.
Involvement in disease
Megacystis-microcolon-intestinal hypoperistalsis syndrome 3 (MMIHS3):
A form of megacystis-microcolon-intestinal hypoperistalsis syndrome, a congenital visceral myopathy primarily affecting females, and characterized by loss of smooth muscle contraction in the bladder and intestine. Affected individuals present at birth with functional obstruction of intestine, microcolon, dilation of bladder, and secondary hydronephrosis. The majority of cases have a fatal outcome due to malnutrition and sepsis, followed by multiorgan failure. MMIHS3 inheritance is autosomal recessive.
More Infomation

Fournier, N., & Fabre, A. (2022). Smooth muscle motility disorder phenotypes: A systematic review of cases associated with seven pathogenic genes (ACTG2, MYH11, FLNA, MYLK, RAD21, MYL9 and LMOD1). Intractable & Rare Diseases Research, 11(3), 113-119.

Tan, Y., Chen, Q., Pan, S., An, W., Xu, H., Xing, Y., & Zhang, J. (2022). LMOD1, an oncogene associated with Lauren classification, regulates the metastasis of gastric cancer cells through the FAK-AKT/mTOR pathway. BMC cancer, 22(1), 474.

Kosiński, K., Malinowski, D., Safranow, K., Dziedziejko, V., & Pawlik, A. (2022). PECAM1, COL4A2, PHACTR1, and LMOD1 Gene Polymorphisms in Patients with Unstable Angina. Journal of Clinical Medicine, 11(2), 373.

Liu, K., Lu, L., Chen, S., Gu, B., Cai, H., Wang, Y., & Cai, W. (2022). Loss‐of‐function variants within LMOD1 actin‐binding site 2 cause pediatric intestinal pseudo‐obstruction by impairing protein stability and actin nucleation. The FASEB Journal, 36(3), e22194.

Qiao, X., Fu, S., Duan, X., Zuo, Y., Zhu, X., Wang, H., & Wang, J. (2021). MYLK, CNN1, TAGLN and LMOD1 identified as potential prognostic biomarkers for bladder cancer using bioinformatics analysis.

Nanda, V., Wang, T., Pjanic, M., Liu, B., Quertermous, T., Miano, J., ... & Miller, C. L. (2018). Regulatory Mechanism of Lmod1 Mediated Predisposition to Coronary Artery Disease. Arteriosclerosis, Thrombosis, and Vascular Biology, 38(Suppl_1), A619-A619.

Nanda, V., Wang, T., Pjanic, M., Liu, B., Nguyen, T., Matic, L. P., ... & Miller, C. L. (2018). Functional regulatory mechanism of smooth muscle cell-restricted LMOD1 coronary artery disease locus. PLoS genetics, 14(11), e1007755.

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

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