Anti Lamin B [BBmLBR 12.F8] Monoclonal Antibody
Invented by Brian Burke
Invented at A*STAR Accelerate Technologies Pte Ltd
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Info
| Catalogue Number | 154119 |
| Applications | IHC IF WB |
| Antigen/Gene or Protein Targets | Lamin B |
| Synonyms | Anti-Lamin B [BBmLBR 12.F8] |
| Reactivity | Human and Mouse |
| Host | Mouse |
| Immunogen UniProt ID | Q3U9G9 |
| Positive Control | Hela, U2OS, C2C12, NIE-115 |
| Molecular Weight (kDa) | Human LBR 70.7 kDa; mouse LBR 71.4 kDa |
| Notes |
Lamin B Receptor (LBR) is a polytopic inner nuclear membrane protein. Its nucleoplasmic domain interacts with components of the nuclear lamina as well as with chromatin, via HP1 (1, 2, 3). LBR also exhibits C14 sterol reductase activity (4). Deficiency of LBR in humans results in Pelget-Huët anomaly featuring hypolobulated granulocyte nuclei (5). Homozygous loss of LBR causes Greenberg skeletal dysplasia a fetal lethal disorder associated with defective cholesterol metabolism (6). 1. Worman, H.J., J. Yuan, G. Blobel, S. D. Georgatos. 1988. A lamin B receptor in the nuclear envelope. Proc Natl Acad Sci U S A. 85(22):853-4. 2. Ye, Q., H. J. Worman. 1994. Primary structure analysis and lamin B and DNA binding of human LBR, an integral protein of the nuclear envelope inner membrane. J Biol Chem. 269(15):11306-11. 3. Ye, Q., H. J Worman. 1996. Interaction between an integral protein of the nuclear envelope inner membrane and human chromodomain proteins homologous to drosophila hp1. J Biol Chem. 271:14653–14656. 4. Tsai, P.L., C. Zhao, E. Turner, C. Schlieker. 2016. The Lamin B receptor is essential for cholesterol synthesis and perturbed by disease-causing mutations. Elife. Jun 23;5. pii: e16011. doi: 10.7554/eLife.16011. 5. Hoffmann, K., C. K. Dreger, A. L. Olins, D. E. Olins, L. D. Shultz, B. Lucke, H. Karl, R. Kaps, D. Müller, A. Vayá, J. Aznar, R. E. Ware, N. Sotelo Cruz, T. H. Lindner, H. Herrmann, A. Reis, K. Sperling. 2002. Mutations in the gene encoding the lamin B receptor produce an altered nuclear morphology in granulocytes (Pelger-Huët anomaly). Nat Genet. 31(4):410-4. 6. Waterham, H.R., J. Koster, P. Mooyer, Gv. Gv. Noort , R. I. Kelley, W. R. Wilcox, R. J Wanders, R. C. Hennekam, J. C, Oosterwijk. 2003. Autosomal recessive HEM/Greenberg skeletal dysplasia is caused by 3 beta-hydroxysterol delta 14-reductase deficiency due to mutations in the lamin B receptor gene. Am J Hum Genet. 72(4):1013-7. 7. Cohen, T.V., K. D Klarmann, K. Sakchaisri, J. P Cooper, D. Kuhns, M. Anver, P. F. Johnson, S. C. Williams, J. R. Keller, C. L. Stewart. 2008. The lamin B receptor under transcriptional control of C/EBPepsilon is required for morphological but not functional maturation of neutrophils. Hum Mol 2 Genet. 17(19):2921-33 |
| Research Area | Metabolism |
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Inventor Information
Inventors
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Brian Burke |
