Product Name
LEMD3, Blocking Peptide
Full Product Name
LEMD3 Peptide
Product Synonym Names
MAN1; MAN1; Inner nuclear membrane protein Man1; LEM domain-containing protein 3; LEM domain containing 3
Product Gene Name
LEMD3 blocking peptide
[Similar Products]
LEMD3 peptide (MBS152635) is used for blocking the activity of LEMD3 antibody (MBS151102)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q9Y2U8
Concentration
200 ug/mL (lot specific)
Buffer
PBS pH 7.2 (10 mM NaH2PO4, 10 mM Na2HPO4, 130 mM NaCl) containing 0.1% bovine serum albumin and 0.02% sodium azide
Location
18 amino acids near the carboxy terminus of human LEMD3.
Preparation and Storage
Store LEMD3 peptide at -20 degree C, stable for one year.
Other Notes
Small volumes of LEMD3 blocking peptide 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.
Applications Tested/Suitable for LEMD3 blocking peptide
Blocking (BL)
Application Notes for LEMD3 blocking peptide
LEMD3 peptide is used for blocking the activity of LEMD3 antibody.
NCBI/Uniprot data below describe general gene information for LEMD3. It may not necessarily be applicable to this product.
NCBI Accession #
NP_055134
[Other Products]
NCBI GenBank Nucleotide #
NM_014319.4
[Other Products]
UniProt Primary Accession #
Q9Y2U8
[Other Products]
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; LEM domain-containing protein 3; integral inner nuclear membrane protein
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: Nuclear envelope; Membrane protein, multi-pass; Membrane protein, integral
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. a nuclear envelope-localized mechanism of inactivating TGF-beta signaling in which MAN1 competes with transcription factors for binding to Smad2 and Smad3 and facilitates their dephosphorylation by PPM1A.
Precautions
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Disclaimer
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