Monoclonal LEMD3; Anti-LEMD3; LEM domain containing 3; MAN1; LEM domain-containing protein 3; Inner nuclear membrane protein Man1; Integral inner nuclear membrane protein; LEMD3; LEMD 3; LEMD-3

For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

IgG1

3H1

Mouse

LEMD3 antibody was purified by affinity chromatography.

Supplied in PBS buffer, pH 7.3, containing 1% BSA, 50% glycerol and 0.02% sodium azide.

500 ug-1 mg/ml (lot specific)

Store at 4 degree C for short term storage. Aliquot and store at -20 degree C for long term storage. Avoid repeated freeze/thaw cycles.

Small volumes of anti-LEMD3 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Mouse monoclonal LEMD3 antibody

Signal Transduction

Flow Cytometry (FC/FACS), Immunofluorescence (IF), Western Blot (WB)

IF: 1:100
FC: 1:100
WB: 1:500

Western Blot analysis of HEK293T cell lysates (5 ug) transfected with either recombinant LEMD3 protein (Right) or empty vector (Left) detected with LEMD3 antibody

Immunofluorescent staining of COS7 cells transiently transfected with recombinant LEMD3 protein using LEMD3 antibody

99,997 Da

LEM domain containing 3

inner nuclear membrane protein Man1

Inner nuclear membrane protein Man1

MAN1??[Similar Products]

MAN1_HUMAN

This locus encodes a LEM domain-containing protein. The encoded protein functions to antagonize transforming growth factor-beta signaling at the inner nuclear membrane. Two transcript variants encoding different isoforms have been found for this gene. Mutations in this gene have been associated with osteopoikilosis, Buschke-Ollendorff syndrome and melorheostosis.[provided by RefSeq, Nov 2009]

MAN1: 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. Defects in LEMD3 are the cause of Buschke-Ollendorff syndrome (BOS); also known as dermatoosteopoikilosis or disseminated dermatofibrosis with osteopoikilosis or dermatofibrosis lenticularis disseminata with osteopoikilosis or osteopathia condensans disseminata. BOS refers to the association of osteopoikilosis with 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. Both 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. Defects in LEMD3 are a cause of melorheostosis (MEL). Melorheostosis is a rare mesenchymal dysplasia and one of the sclerosing bone disorders. It is caused by a developmental error, with a sclerotomal distribution, frequently involving one limb. It may be asymptomatic, but pain, stiffness with limitation of motion, leg-length discrepancy and limb deformity may occur.

Protein type: Membrane protein, multi-pass; Membrane protein, integral; Nuclear envelope

Chromosomal Location of Human Ortholog: 12q14

Cellular Component: membrane; integral to membrane; nuclear inner membrane; integral to nuclear inner membrane

Molecular Function: protein binding; DNA binding; nucleotide binding

Biological Process: blood vessel endothelial cell migration during intussusceptive angiogenesis; negative regulation of activin receptor signaling pathway; regulation of cell cycle; negative regulation of transforming growth factor beta receptor signaling pathway; nuclear organization and biogenesis; negative regulation of BMP signaling pathway

Disease: Buschke-ollendorff Syndrome; Melorheostosis, Isolated

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