Cellular and Molecular Life Sciences CMLS

, Volume 58, Issue 12–13, pp 1741–1747 | Cite as

Inner nuclear membrane proteins: functions and targeting

  • L. Holmer
  • H.J. Worman

Abstract:

We summarize the properties of integral membrane proteins that reside in the inner nuclear membrane, including lamin B receptor (LBR), lamina-associated polypeptide (LAP) 1, LAP2, emerin, MAN1 and nurim. Most of these proteins interact with lamins and chromatin. Some data also suggest more speculative functions such as gene regulation and possibly sterol metabolism. Mutations in emerin and nuclear lamins have been associated with muscular dystrophies and lipodystrophy, raising new questions about the functions of inner nuclear membrane proteins. Integral proteins of the inner nuclear membrane are synthesized on the rough endoplasmic reticulum (ER) and reach the inner nuclear membrane by lateral diffusion in the connected ER and nuclear envelope membranes. Associations with nuclear ligands retain them in the inner nuclear membrane. Further investigation of the functions and targeting of inner nuclear membrane proteins are needed to determine how they are involved in human disease.

Key words: Nuclear envelope; nuclear lamina; Emery-Dreifuss muscular dystrophy; limb-girdle muscular dystrophy; Dunnigan-type partial lipodystrophy; intracellular protein trafficking; lamin B receptor; lamina-associated polypeptide; nurim. 

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Copyright information

© Birkhäuser Verlag, 2001

Authors and Affiliations

  • L. Holmer
    • 1
  • H.J. Worman
    • 1
  1. 1.Departments of Medicine and of Anatomy and Cell Biology, Columbia University College of Physicians and Surgeons, 630 W 168th Street, New York (New York 10032, USA), Fax: + 212 305 6443, e-mail: hjw14@columbia.eduUS

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