Cancer Research Technology
Log in Register
Menu

Anti Lamin B [BBmLBR 12.F8] Monoclonal Antibody

Invented by Brian Burke

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

References

There are 0 reference entries for this reagent.

References: 0 entry

There is no reference for this reagent yet, feel free to use the button below to suggest one.

Add a reference

References: 0 entry

There is no reference for this reagent yet, feel free to use the button below to suggest one.

Add a reference

Inventor Information