A-type lamin networks in light of laminopathic diseases.
Lamins are major structural components of the lamina providing mechanical support for the nuclear envelope in vertebrates. A subgroup of lamins, the A-type lamins, are only expressed in differentiated cells and serve important functions both at the nuclear envelope and in the nucleoplasm in higher order chromatin organization and gene regulation. Mutations in A-type lamins cause a variety of diseases from muscular dystrophy and lipodystrophy to systemic diseases such as premature ageing syndromes. The molecular basis of these diseases is still unknown. Here we summarize known interactions of A-type lamins with components of the nuclear envelope and the nucleoplasm and discuss their potential involvement in the etiology and molecular mechanisms of the diseases. Lamin binding partners involve chromatin proteins potentially involved in higher order chromatin organization, transcriptional regulators controlling gene expression during cell cycle progression, differentiation and senescence, and several enzymes involved in a multitude of functions.
Vlcek
Sylvia
S
Max. F. Perutz Laboratories, Department of Medical Biochemistry, Medical University of Vienna, Dr. Bohr-Gasse 9, A-1030 Vienna, Austria.
Foisner
Roland
R
D002843:Chromatin / Q000235:genetics / Q000378:metabolism; D005786:Gene Expression Regulation; D006801:Humans; D034904:Lamin Type A / Q000235:genetics / Q000378:metabolism; D034882:Lamins / Q000737:chemistry / Q000235:genetics* / Q000378:metabolism*; D008060:Lipodystrophy / Q000235:genetics*; D008958:Models, Molecular; D011487:Protein Conformation
D002843:Chromatin; D034904:Lamin Type A; D034882:Lamins