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lamins

Sunday 13 July 2003

Lamins constitute a family of polypeptides composing the proteinaceous layer apposed to the inner nuclear membrane. In mammals, 3 lamins, A (LMNA), B (LMNB), and C (encoded by LMNA), have been described, with molecular weights ranging from 60,000 to 78,000. Lamins A and C but not lamin B have close sequence homology. Lamins can be classified with the intermediate filament (IF) polypeptides.

Members

LMNA lamin-A MIM.150330
LMNB1 lamin-B1 MIM.150340
LMNB2 lamin-B2 MIM.150341
LMNC lamin-C MIM.150330

Pathology (laminopathies)

LMNA lamin-A autosomal dominant Emery-Dreifuss muscular dystrophy MIM.181350
LMNA lamin-A autosomal dominant dilated cardiomyopathy and conduction system disease MIM.115200
LMNA lamin-A partial lipodystrophy type 2 MIM.151660
LMNA lamin-A mandibuloacral dysplasia with lipodystrophy MIM.248370
LMNA lamin-A limb-girdle muscular dystrophy type 1B MIM.159001
LMNA lamin-A axonal Charcot-Marie-Tooth Disease type 2B1 MIM.605588
LMNA lamin-A Hutchinson-Gilford progeria syndrome MIM.176670
LMNA lamin-A LDHCP syndrome MIM.608056
LMNA lamin-A variant in obesity-related traits
LMNB1 lamin-B1 no disease MIM.150340
LMNB2 lamin-B2 no disease MIM.150341
LMNC lamin-C no disease MIM.150330

See also

- progeria

References

- Broers JL, Hutchison CJ, Ramaekers FC. Laminopathies. J Pathol. 2004 Nov;204(4):478-88. PMID: 15495262

- Worman HJ, Courvalin JC. The nuclear lamina and inherited disease. Trends Cell Biol. 2002 Dec ;12(12):591-8. PMID : 12495848

- Burke B, Stewart CL. Life at the edge : the nuclear envelope and human disease. Nat Rev Mol Cell Biol. 2002 Aug ;3(8):575-85. PMID : 12154369

- Mounkes LC, Stewart CL. Aging and nuclear organization: lamins and progeria. Curr Opin Cell Biol. 2004 Jun;16(3):322-7. PMID: 15145358

- Herrmann H, Foisner R. Intermediate filaments: novel assembly models and exciting new functions for nuclear lamins. Cell Mol Life Sci. 2003 Aug;60(8):1607-12. PMID: 14504651

- Burke B, Stewart CL. Life at the edge: the nuclear envelope and human disease. Nat Rev Mol Cell Biol. 2002 Aug;3(8):575-85. PMID: 12154369

- Moir RD, Spann TP. The structure and function of nuclear lamins: implications for disease. Cell Mol Life Sci. 2001 Nov;58(12-13):1748-57. PMID: 11766876