The outer-most part of the nucleus is the nuclear envelope. This consists of a double lipid bilayer. The nuclear envelope completely surrounds the nucleoplasm.

The double-membrane acts as a barrier to water-soluble substances. Clusters of membrane bound proteins form a basket-like apparatus called nuclear pore complex (NCP). These apparati allow the passage of RNA and proteins to flow in and out of the nucleus. The structural strength of the nucleus comes from a layer of nuclear lamina that lies just inside of the nuclear membrane.

Struture

The nuclear envelope is composed of the nuclear lamina, the nuclear pore complexes, and the nuclear membranes.

 The nuclear lamina is a discontinuous structure that occupies only a fraction of the nuclear periphery, and at some points, the inner nuclear membrane may interact directly with the chromatin.

 The nuclear membranes can be divided into 3 morphologically distinct but interconnected domains: the outer nuclear membrane, the inner nuclear membrane, and the nuclear pore membrane.

• The inner nuclear membrane is adjacent to the nuclear lamina, a meshwork of intermediate filament proteins termed lamins.
• Several integral proteins of the nuclear envelope inner membrane that may be associated with the lamina and the chromatin have been identified. The first was a protein called lamin B receptor (LBR) that binds in vitro to lamin B.

Features

 nuclear membrane proteins (nuclear envelope proteins)
 nuclear pore complexes (NPCs)

Pathogy

 laminopathies

• pathology of lamins
• pathology of lamin receptors

Pathology (nuclear envelopathies)

 emerin deficiency results in X-linked Emery-Dreifuss muscular dystrophy (EDMD).
 lamin A/C mutations cause

• autosomal-dominant form of EDMD
• limb-girdle muscular dystrophy with atrioventricular conduction disturbances (type 1B)
• hypertrophic cardiomyopathy and Dunnigan-type familial partial lipodystrophy
• progeria

References

 Sazer S. Nuclear envelope: nuclear pore complexity. Curr Biol. 2005 Jan 11;15(1):R23-6. PMID: #15649347#

 Mosammaparast N, Pemberton LF. Karyopherins: from nuclear-transport mediators to nuclear-function regulators. Trends Cell Biol. 2004 Oct;14(10):547-56. PMID: #15450977#

 Mattout-Drubezki A, Gruenbaum Y. Dynamic interactions of nuclear lamina proteins with chromatin and transcriptional machinery. Cell Mol Life Sci. 2003 Oct;60(10):2053-63. PMID: #14618255#

 Morris GE. The role of the nuclear envelope in Emery-Dreifuss muscular dystrophy. Trends Mol Med. 2001 Dec;7(12):572-7. PMID: #11733221#

 Vlcek S, Dechat T, Foisner R. Nuclear envelope and nuclear matrix: interactions and dynamics. Cell Mol Life Sci. 2001 Nov;58(12-13):1758-65. PMID: #11767745#

 Salina D, Bodoor K, Enarson P, Raharjo WH, Burke B. Nuclear envelope dynamics. Biochem Cell Biol. 2001;79(5):533-42. PMID: #11716295#