Full Product Name
LEMD3 Antibody
Product Synonym Names
LEM domain containing 3, MAN1
Product Gene Name
anti-LEMD3 antibody
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Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Species Reactivity
Human, Mouse
Specificity
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
Purity/Purification
Affinity chromatography purified via peptide column
Form/Format
Supplied in PBS containing 0.02% sodium azide.
Immunogen Description
Raised against an 18 amino acid peptide near the carboxy terminus of human LEMD3.
Preparation and Storage
Can be stored at -20 degree C, stable for 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.
Other Notes
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.
Related Product Information for
anti-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.
Product Categories/Family for anti-LEMD3 antibody
Total protein Ab
Applications Tested/Suitable for anti-LEMD3 antibody
ELISA (EIA), Western Blot (WB)
Western Blot (WB) of anti-LEMD3 antibody
Western blot analysis of LEMD3 in human colon tissue lysate with LEMD3 antibody at 1 ug/mL.
NCBI/Uniprot data below describe general gene information for LEMD3. It may not necessarily be applicable to this product.
NCBI Accession #
NP_055134
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NCBI GenBank Nucleotide #
NM_014319.4
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UniProt Secondary Accession #
Q9NT47; Q9NYA5[Other Products]
UniProt Related Accession #
Q9Y2U8[Other Products]
Molecular Weight
99,997 Da
NCBI Official Full Name
inner nuclear membrane protein Man1 isoform 1
NCBI Official Synonym Full Names
LEM domain containing 3
NCBI Official Symbol
LEMD3??[Similar Products]
NCBI Official Synonym Symbols
MAN1
??[Similar Products]
NCBI Protein Information
inner nuclear membrane protein Man1
UniProt Protein Name
Inner nuclear membrane protein Man1
UniProt Synonym Protein Names
LEM domain-containing protein 3
Protein Family
Inner nuclear membrane protein
UniProt Gene Name
LEMD3??[Similar Products]
UniProt Synonym Gene Names
MAN1??[Similar Products]
UniProt Entry Name
MAN1_HUMAN
NCBI Summary for LEMD3
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]
UniProt Comments for LEMD3
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
Research Articles on LEMD3
1. Data indicate that the inner nuclear membrane protein MAN1 directly binds the transcription activator BMAL1 promoter and enhances its transcription.
Precautions
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