Nuclear Lamins: Biology and Roles in Cell Function and Dysregulation.

Nuclear Lamins: Biology and Roles in Cell Function and Dysregulation. Recent Pat Endocr Metab Immune Drug Discov. 2015 Oct 9; Authors: Khadija SG, Chen F, Hadden T, Commissaris RL, Kowluru A Abstract Nuclear lamins, namely lamins A, B and C, surround the nucleoplasmic contents in a mesh-like network called the nuclear lamina. These intermediate filaments provide a structural framework to the nuclear envelope (NE), play a role in arrangement of the chromatin within the nucleus, in DNA replication and also participate in DNA damage repair. In order for lamins to be involved in these important nuclear processes and to be functionally active they undergo a series of post-translational modifications (farnesylation, endoproteolytic cleavage, carboxylmethylation etc.), of which farnesylation is the most studied. Improper farnesylation of lamin proteins, especially lamin A, leads to a number of diseases affecting the striated muscle (e.g. Emery-Dreifuss Muscular Dystrophy, Dilated Cardiomyopathy), adipose tissue (e.g. Dunnigan-type familial partial lipodystrophy) and could result to abnormal senescence and growth deformities (e.g. Progeria syndrome); these are referred to as laminopathies. Despite the existing literature and evidence regarding functioning of lamins and diseases associated with abnormal lamin processing, a lot remains to be understood in regards to lamin biology and their role as potential therapeutic targets. In this brief r...
Source: Recent Patents on Endocrine, Metabolic and Immune Drug Discovery - Category: Endocrinology Tags: Recent Pat Endocr Metab Immune Drug Discov Source Type: research