LEMD3; MAN1; MAN1; Inner nuclear membrane protein Man1; LEM domain-containing protein 3; LEM domain containing 3

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

Polyclonal

IgG

Rabbit

At least three isoforms of LEMD3 are known to exist; this antibody will detect all isoforms. LEMD3 antibody is predicted to not cross-react with LEMD1 and LEMD2

LEMD3 Antibody is affinity chromatography purified via peptide column.

Liquid

1 mg/mL (lot specific)

LEMD3 antibody can be stored at 4 degree C for three months and -20 degree C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

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.

LEMD3 Antibody: LEMD3 (LEM domain-containing 3) is a lem-domain containing protein, also known as MAN1, whose loss of function results in disorders characterized by osteopoikilosis, Buschke-Ollendorff syndrome, and melorheostosis. LEMD3 is an inner nuclear membrane protein that interacts with BMP and activin-TGF-beta receptor-activated Smads and antagonizes TGF-beta signaling in human cells.

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

LEMD3 antibody can be used for detection of LEMD3 by Western blot at 1 mug/mL. Antibody can also be used for immunohistochemistry starting at 2.5 mug/mL. For immunofluorescence start at 5 mug/mL.

Western blot analysis of LEMD3 in human colon tissue lysate with LEMD3 antibody at 1 μg/mL.

Immunohistochemistry of LEMD3 in human colon tissue with LEMD3 antibody at 2.5 μg/mL.

Immunofluorescence of LEMD3 in human colon tissue with LEMD3 antibody at 5 μg/mL.

99,997 Da

LEM domain containing 3

inner nuclear membrane protein Man1; LEM domain-containing protein 3; integral inner nuclear membrane protein

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, integral; Nuclear envelope; Membrane protein, multi-pass

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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