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Lamins, laminopathies and disease mechanisms: Possible role for proteasomal degradation of key regulatory proteins

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Abstract

Lamins are major structural proteins of the nucleus and are essential for nuclear integrity and organization of nuclear functions. Mutations in the human lamin genes lead to highly degenerative genetic diseases that affect a number of different tissues such as muscle, adipose or neuronal tissues, or cause premature ageing syndromes. New findings on the role of lamins in cellular signalling pathways, as well as in ubiquitin-mediated proteasomal degradation, have given important insights into possible mechanisms of pathogenesis.

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Abbreviations

ATR:

ATM-and-Rad3-related

BAF:

barrier-to-autointegration factor

Cdk1:

cyclin-dependent kinase 1

CMT:

Charcot-Marie-Tooth disorder

DCM:

dilated cardiomyopathy

EMD:

Emery-Dreifuss muscular dystrophy

FPLD:

familial partial lipodystrophy

HGPS:

Hutchinson-Gilford progeria syndrome

HP1α:

heterochromatin protein 1α

Ig:

immunoglobulin

LAP:

lamin-associated-polypeptide

LEM:

LAP, emerin, MAN1

LGMD:

limb girdle muscular dystrophy

MAD:

mandibuloacral dysplasia

MAPK:

mitogen-activated protein kinase

PCNA:

proliferating cell nuclear antigen

pRb:

retinoblastoma protein

SREBP1:

sterol response element binding protein 1

SUN:

Sad1/UNC-84 homology

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Acknowledgements

We apologize to those whose references have not been cited due to space restrictions. VKP is a recipient of the JC Bose National Fellowship from the Department of Science and Technology. PC was supported by a senior research fellowship from the Council of Scientific and Industrial Research. Research in VKP’s laboratory has been supported by the Council of Scientific and Industrial Research, Department of Biotechnology and Department of Science and Technology.

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  1. Centre for Cellular and Molecular Biology (CSIR), Hyderabad, 500 007, India

    Veena K Parnaik, Pankaj Chaturvedi & Bh Muralikrishna

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  1. Veena K Parnaik
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Correspondence to Veena K Parnaik.

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[Parnaik VK, Chaturvedi P and Muralikrishna Bh 2011 Lamins, laminopathies and disease mechanisms: Possible role for proteasomal degradation of key regulatory proteins. J. Biosci. 36 471–479] DOI 10.1007/s12038-011-9085-2

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Parnaik, V.K., Chaturvedi, P. & Muralikrishna, B. Lamins, laminopathies and disease mechanisms: Possible role for proteasomal degradation of key regulatory proteins. J Biosci 36, 471–479 (2011). https://doi.org/10.1007/s12038-011-9085-2

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