Full Product Name
LBR Rabbit Polyclonal
Product Gene Name
anti-LBR antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Species Reactivity
Human, Mouse, Rat
Purity/Purification
>=95% as determined by SDS-PAGE
Immunogen Affinity Purified
Immunogen
Lamin B receptor
Preparation and Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of anti-LBR 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.
Applications Tested/Suitable for anti-LBR antibody
ELISA (EIA), Western Blot (WB), Immunofluorescence (IF), Immunoprecipitation (IP)
NCBI/Uniprot data below describe general gene information for LBR. It may not necessarily be applicable to this product.
NCBI Accession #
NP_919424.1
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NCBI GenBank Nucleotide #
NM_194442.2
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UniProt Secondary Accession #
Q14740; Q53GU7; Q59FE6; B2R5P3[Other Products]
UniProt Related Accession #
Q14739[Other Products]
Molecular Weight
70,703 Da
NCBI Official Full Name
lamin-B receptor
NCBI Official Synonym Full Names
lamin B receptor
NCBI Official Symbol
LBR??[Similar Products]
NCBI Official Synonym Symbols
PHA; LMN2R; TDRD18; DHCR14B
??[Similar Products]
NCBI Protein Information
lamin-B receptor
UniProt Protein Name
Lamin-B receptor
UniProt Synonym Protein Names
Integral nuclear envelope inner membrane protein; LMN2R
Protein Family
Lamin-B receptor
UniProt Gene Name
LBR??[Similar Products]
UniProt Entry Name
LBR_HUMAN
NCBI Summary for LBR
The protein encoded by this gene belongs to the ERG4/ERG24 family. It localized in the nuclear envelope inner membrane and anchors the lamina and the heterochromatin to the membrane. It may mediate interaction between chromatin and lamin B. Mutations of this gene has been associated with autosomal recessive HEM/Greenberg skeletal dysplasia. Alternative splicing occurs at this locus and two transcript variants encoding the same protein have been identified. [provided by RefSeq, Jul 2008]
UniProt Comments for LBR
LBR: Anchors the lamina and the heterochromatin to the inner nuclear membrane. Defects in LBR are a cause of Pelger-Huet anomaly (PHA). PHA is an autosomal dominant inherited abnormality of neutrophils, characterized by reduced nuclear segmentation and an apparently looser chromatin structure. Heterozygotes show hypolobulated neutrophil nuclei with coarse chromatin. Presumed homozygous individuals have ovoid neutrophil nuclei, as well as varying degrees of developmental delay, epilepsy, and skeletal abnormalities. Defects in LBR are the cause of hydrops-ectopic calcification-moth-eaten skeletal dysplasia (HEM); also known as Greenberg skeletal dysplasia. HEM is a rare autosomal recessive chondrodystrophy characterized by early in utero lethality and, therefore, considered to be nonviable. Affected fetuses typically present with fetal hydrops, short- limbed dwarfism, and a marked disorganization of chondro-osseous calcification and may present with polydactyly and additional nonskeletal malformations. Defects in LBR may be a cause of Reynolds syndrome (REYNS). It is a syndrome specifically associating limited cutaneous systemic sclerosis and primary biliray cirrhosis. It is characterized by liver disease, telangiectasia, abrupt onset of digital paleness or cyanosis in response to cold exposure or stress (Raynaud phenomenon), and variable features of scleroderma. The liver disease is characterized by pruritis, jaundice, hepatomegaly, increased serum alkaline phosphatase and positive serum mitochondrial autoantibodies, all consistent with primary biliary cirrhosis. Belongs to the ERG4/ERG24 family.
Protein type: Membrane protein, integral; DNA-binding; Membrane protein, multi-pass
Chromosomal Location of Human Ortholog: 1q42.1
Cellular Component: integral to endoplasmic reticulum membrane; integral to membrane; integral to nuclear inner membrane; membrane; nuclear envelope; nuclear membrane
Molecular Function: delta14-sterol reductase activity; DNA binding; lamin binding; oxidoreductase activity, acting on the CH-CH group of donors; protein binding
Biological Process: cholesterol biosynthetic process; sterol biosynthetic process
Disease: Greenberg Dysplasia; Pelger-huet Anomaly; Reynolds Syndrome
Research Articles on LBR
1. Lamin B receptor mRNA expression was directly associated with tumor grade in breast cancer patients(grade 1 vs. grade 3 - 0.00 vs. 0.00; p = 0.0479) and Nottingham Prognostic Index (NPI1 vs. NPI3 - 0.00 vs. 0.00; p = 0.0551).
Precautions
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Disclaimer
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