mRNAs in the protective mechanism of neurons during Progeria

Abstract

Progeria syndrome or Hutchinson-Gilford is a rare, fatal and premature aging disease that manifests early in life. The purpose of this paper is to present the action of microRNA's in the protective mechanism of neurons during progeria in association with the clinical manifestations of the disease. For this, a literature search was performed with revision in technical and scientific papers. The diagnosis is based on clinical manifestations of the patients, such as scleroderma, alopecia, atherosclerosis, cardiovascular disease, among others, while the existing treatment is based on drugs which are intended only to minimize the symptoms. The causative gene is called progeria LMNA, responsible for the production of an abnormal protein called progerina, which accumulates in the cells causing significant molecular defects, such as premature cell death. Although the abnormalities that affect tissue and organ of individuals affected by the disease, its memory and motor function remain intact thanks to a molecular mechanism involved in protecting neurons microRNA, miR-9, which is expressed only by nerve cells and acts as inhibitor in the expression of LMNA gene. According to the research purpose, the result obtained was satisfactory, since progerina is expressed by all cell types except for the brain, thus saving the reasoning ability of patients with the syndrome. Therefore, we conclude that the progeria allows these children enjoy the simplest to the most complex functions at the same time that the typical signs and symptoms of the disease will progress considerably, to lead them todeath.