Mouse Anti-LEMD3 Recombinant Antibody (4E1) (CBMAB-L1098-YJ)

Provided herein is a Mouse monoclonal antibody, which binds to Lem Domain Containing 3 (LEMD3). The antibody can be used for immunoassay techniques, such as FC, ICC, IF, IHC-P, WB.
See all LEMD3 antibodies

Datasheet

Summary

Host Animal

Mouse

Specificity

Human

Clone

4E1

Antibody Isotype

IgG2b

Application

FC, ICC, IF, IHC-P, WB

Basic Information

Immunogen

Full length human recombinant protein of human LEMD3 (NP_055134) produced in HEK293T cell.

Specificity

Human

Antibody Isotype

IgG2b

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!]

Buffer

PBS, pH 7.3, 1% BSA, 50% Glycerol

Preservative

0.02% Sodium Azide

Storage

Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name

LEM DOMAIN CONTAINING 3

Introduction

LEMD3 functions to antagonize transforming growth factor-beta signaling at the inner nuclear membrane. Two transcript variants encoding different isoforms have been abserved for this gene. Mutations in this gene have been associated with osteopoikilosis, Buschke-Ollendorff syndrome and melorheostosis. Diseases associated with LEMD3 include Buschke-Ollendorff Syndrome and Melorheostosis With Osteopoikilosis. Among its related pathways are Cell Cycle, Mitotic and Nuclear Envelope Reassembly. Gene Ontology (GO) annotations related to this gene include nucleotide binding. An important paralog of LEMD3 is LEMD2. It can function as a specific repressor of TGF-beta, activin, and BMP signaling through its interaction with the R-SMAD proteins. Antagonizes TGF-beta-induced cell proliferation arrest.

Entrez Gene ID

23592

UniProt ID

Q9Y2U8

Alternative Names

MAN1

Function

Can function as a specific repressor of TGF-beta, activin, and BMP signaling through its interaction with the R-SMAD proteins. Antagonizes TGF-beta-induced cell proliferation arrest.

Biological Process

Negative regulation of activin receptor signaling pathwayManual Assertion Based On ExperimentIDA:UniProtKB
Negative regulation of BMP signaling pathwayManual Assertion Based On ExperimentIDA:UniProtKB
Negative regulation of transforming growth factor beta receptor signaling pathwayManual Assertion Based On ExperimentIDA:UniProtKB
Nuclear envelope organizationManual Assertion Based On ExperimentIBA:GO_Central
Regulation of intracellular signal transductionIEA:InterPro

Cellular Location

Nucleus inner membrane

Involvement in disease

Buschke-Ollendorff syndrome (BOS):
A disease characterized by osteopoikilosis and disseminated connective-tissue nevi. Osteopoikilosis is a skeletal dysplasia characterized by a symmetric but unequal distribution of multiple hyperostotic areas in different parts of the skeleton. Elastic-type nevi (juvenile elastoma) and collagen-type nevi (dermatofibrosis lenticularis disseminata) have been described in BOS. Skin or bony lesions can be absent in some family members, whereas other relatives may have both.

References

Xu, Z., Yang, C., & Xue, R. (2021). Buschke‐Ollendorff syndrome with LEMD3 germline stopgain mutation p. R678* presenting as multiple subcutaneous nodules with mucin deposition. Journal of Cutaneous Pathology, 48(1), 77-80.

Ji, D., Chen, G. F., Wang, J. C., Ji, S. H., Wu, X. W., Lu, X. J., ... & Li, J. T. (2020). Hsa_circ_0070963 inhibits liver fibrosis via regulation of miR-223-3p and LEMD3. Aging (Albany NY), 12(2), 1643.

Frost, M., Rahbek, E. T., Ejersted, C., Høilund-Carlsen, P. F., Bygum, A., Thomsen, J. S., ... & Frederiksen, A. L. (2020). Modeling-based bone formation transforms trabeculae to cortical bone in the sclerotic areas in Buschke-Ollendorff syndrome. A case study of two females with LEMD3 variants. Bone, 135, 115313.

Llano, M. G. C., Mora, J., Torner, F., Inarejos, E., & Cruz, O. (2020). Osteopoikilosis with germline LEMD3 mutation mimicking bone metastases in a girl with a concurrent secreting mixed germ cell tumor. Journal of Pediatric Hematology/Oncology, 42(3), e185-e187.

Elmaoğulları, S., Yıldız, A. E., Demir, S., Gürkan, H., & Uçaktürk, S. A. (2019). A novel LEMD3 pathogenic variant in a son and mother with osteopoikilosis. The Turkish Journal of Pediatrics, 61(4), 594-598.

Silov, G., Erdoğan, Z., Erdoğan, M., Özdal, A., Gençer, H., Akalın, T., & Karaçavuş, S. (2018). Tc-99m MDP bone SPECT/CT findings of a patient detected with a new mutation in LEMD3 gene: a case of osteopoikilosis. Molecular Imaging and Radionuclide Therapy, 27(1), 48.

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Protocols

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)
Proteogenomic
Other Protocols

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Isotype control

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

Custom Antibody Labeling

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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