Lamin B2

IgG1

LN43 reacts with an epitope located in the Cterminal part of lamin B2. LN43 is suitable for immunohistochemistry on frozen sections. immunoblotting and flow cytometry. Optimal antibody dilution should be determined by titration; recommended range is 1:100 ? 1:200 for flow cytometry. and for immunohistochemistry with avidin-biotinylated horseradish peroxidase complex (ABC) as detection reagent. and 1:100 ? 1:1000 for immunoblotting applications. Product

Lamins are components of the nuclear lamina a fibrous layer on the nucleoplasmic side of the inner nuclear membrane which is thought to provide a framework for the nuclear envelope and may also interact with chromatin.

Nucleus inner membrane; Lipid-anchor; Nucleoplasmic side.

Defects in LMNB2 are a cause of partial acquired lipodystrophy (APL) [MIM:608709]; also called Barraquer-Simons syndrome. APL is a rare childhood disease characterized by loss of subcutaneous fat from the face and trunk. Fat deposition on the pelvic girdle and lower limbs is normal or excessive. Most frequently onset between 5 and 15 years of age. Most affected subjects are females and some show no other abnormality but many develop glomerulonephritis diabetes mellitus hyperlipidaemia and complement deficiency. Mental retardation in some cases. APL is a sporadic disorder of unknown aetiology.

Belongs to the intermediate filament family. 1. Bridger. J. M. Kill. I. R. O\' Farrell. M. and Hutchison. C. J. (1993). Internal lamin structures within G1 nuclei of human dermal fibroblasts. J Cell Sci 104. 297-306. 2. Jenkins. H. Hö lman. T. Lyon. C. Lane. B. Stick. R. and Hutchison. C. J. (1993). Nuclei that lack a lamina accumulate karyophilic proteins and assemble a nuclear matrix. J Cell Sci 106. 275-285. 3. Hozak. P. Sasseville. A. M. Raymond. Y. and Cook. P. R. (1995). Lamin proteins form an internal nucleoskeleton as well as a peripheral lamina in human cells. J Cell Sci 108. 635-44. 5. Machiels. B. M. Zorenc. A. H. Endert. J. M. Kuijpers. H. J. van Eys. G. J. Ramaekers. F. C. and Broers. J. L. (1996). An alternative splicing product of the lamin A/C gene lacks exon 10. J Biol Chem 271. 9249-53. 6. Machiels. B. M. Ramaekers. F. C. Kuijpers. H. J. Groenewoud. J. S. Oosterhuis. J. W. and Looijenga. L. H. (1997). Nuclear lamin expression in normal testis and testicular germ cell tumours of adolescents and adults. J Pathol 182. 197-204. 7. Jansen. M. P. Machiels. B. M. Hopman. A. H. Broers. J. L. Bot. F. J. Arends. J. W. Ramaekers. F. C. and Schouten. H. C. (1997). Comparison of A and B-type lamin expression in reactive lymph nodes and nodular sclerosing Hodgkin\' s disease. Histopathology 31. 304-12.