Premature aging of the body - the role of laminopathy

Abstract

Aging is a process, that went off inevitable and it is associated with the accumulation of macromolecular damage, genomic instability, and loss of heterochromatin. All these changes conduct to deterioration function of stem cells and reducing the ability to regenerate tissues. Current views on the structure and function of the cell nucleus in a normal or pathological cell are focused on the nuclear envelope, and especially on the lamins located in the inner nuclear membrane. The main component of the nuclear lamina is lamins belonging to intermediate filaments. Disorders of their functions, as a result of mutations, have serious health consequences, including leading to premature aging. It was found that the abnormal shape of the cell nucleus is a hallmark of laminopathy. These diseases are relatively rare and include HGSP progeria syndrome, congenital muscular dystrophy, dilated cardiomyopathy (congestive cardiomyopathy, hyperaemic cardiomyopathy), restrictive dermopathy, and familial partial lipodystrophy. An expression of the indispensable role of laminae in many fundamental cellular processes is the fact that defects in these proteins very often underlie a variety of disease states, collectively known as laminopathies. Progeroid syndromes are hereditary diseases with clinical signs similar to aging but prematurely. The well-known disorders of accelerated aging include, among others, Parkinson’s disease or Alzheimer’s disease. Because premature aging syndromes (progeroid syndromes), including progeria, mimic many of the hallmarks of human aging, research into these conditions has proven to be very useful not only for identifying underlying causal mechanisms and identifying treatments for these pathologies but also for studying aging physiological. The subject appears to be perspective and development. This paper is an introduction to the considerations and the aim of this study is to present the role of laminopathy in premature aging processes and paying attention to molecular models of laminopathy.

Keywords

• laminopathies
• progeria
• premature aging
• lamines
• progeroid syndromes