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Lamin B receptor (LBR) is a polytopic membrane protein residing in the inner nuclear membrane in association with the nuclear lamina. We demonstrate that human LBR is essential for cholesterol synthesis. LBR mutant derivatives implicated in Greenberg skeletal dysplasia or Pelger-Huët anomaly fail to rescue the cholesterol auxotrophy of a LBR-deficient human cell line, consistent with a loss-of-function mechanism for these congenital disorders. These disease-causing variants fall into two classes: point mutations in the sterol reductase domain perturb enzymatic activity by reducing the affinity for the essential cofactor NADPH, while LBR truncations render the mutant protein metabolically unstable, leading to its rapid degradation at the inner nuclear membrane. Thus, metabolically unstable LBR variants may serve as long-sought-after model substrates enabling previously impossible investigations of poorly understood protein turnover mechanisms at the inner nuclear membrane of higher eukaryotes.
Pubmed ID: 27336722 RIS Download
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Public registry of antibodies with unique identifiers for commercial and non-commercial antibody reagents to give researchers a way to universally identify antibodies used in publications. The registry contains antibody product information organized according to genes, species, reagent types (antibodies, recombinant proteins, ELISA, siRNA, cDNA clones). Data is provided in many formats so that authors of biological papers, text mining tools and funding agencies can quickly and accurately identify the antibody reagents they and their colleagues used. The Antibody Registry allows any user to submit a new antibody or set of antibodies to the registry via a web form, or via a spreadsheet upload.
View all literature mentionsCell line HEK293T is a Transformed cell line with a species of origin Homo sapiens (Human)
View all literature mentionsThis monoclonal targets Lamin A + C
View all literature mentionsThis polyclonal targets LMNB1
View all literature mentionsThis monoclonal targets beta Actin
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View all literature mentionsThis polyclonal targets Calnexin - ER membrane marker antibody
View all literature mentionsThis monoclonal targets Lamin B Receptor
View all literature mentionsThis polyclonal targets Lamin B Receptor antibody
View all literature mentionsThis polyclonal targets Human hnRNP A2B1
View all literature mentionsThis monoclonal targets hnRNP A1
View all literature mentionsThis monoclonal targets Nuclear Pore Complex Proteins antibody [Mab414] - ChIP Grade
View all literature mentionsCell line HeLa is a Cancer cell line with a species of origin Homo sapiens
View all literature mentionsCell line HEK293T/17 is a Transformed cell line with a species of origin Homo sapiens (Human)
View all literature mentionsCell line HFF-1 is a Finite cell line with a species of origin Homo sapiens (Human)
View all literature mentionsThis polyclonal targets Calnexin - ER membrane marker antibody
View all literature mentionsThis polyclonal targets Human hnRNP A2B1
View all literature mentionsThis monoclonal targets hnRNP A1
View all literature mentionsThis monoclonal targets Nuclear Pore Complex Proteins antibody [Mab414] - ChIP Grade
View all literature mentionsThis monoclonal targets Lamin A + C
View all literature mentionsThis polyclonal targets LMNB1
View all literature mentionsThis monoclonal targets alpha-Tubulin
View all literature mentionsThis polyclonal targets Lamin B Receptor antibody
View all literature mentionsThis monoclonal targets beta Actin
View all literature mentionsThis monoclonal targets Lamin B Receptor
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