Can a mixture of micronutrients delay the onset and progression of Hutchinson-Gilford Progeria syndrome?
1 Engage Global, Inc, 245 El Faisan Drive, San Rafael, CA 94903.
2 Center for Occupational and Environmental Health, University of California, Irvine, CA 92697.
Review
World Journal of Biology Pharmacy and Health Sciences, 2022, 10(03), 073–081.
Article DOI: 10.30574/wjbphs.2022.10.3.0081
Publication history:
Received on 14 May 2022; revised on 22 June 2022; accepted on 24 June 2022
Abstract:
Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare autosomal dominant genetic disorder of accelerated aging caused by a point mutation in LMNA gene coding for a nuclear protein Lamin A which maintains normal structure and function of the nucleus. Mutated Lamin A protein undergoes a series of enzymatic modifications including farnesylation and prenylation to form a toxic protein progerin that alters nuclear structure and function. HGPS disease begins in utero; however, infants appear normal at birth with some features such as circumoral pallor. The expression of progerin is low in undifferentiated cells which may account for the fewer symptoms of progeria. In about 9-12 months, the levels of progerin in cells become high enough to produce nuclear structure abnormalities leading to cellular and molecular damages characteristic of HGPS. The average age of these patients is 14.6 years. Some therapeutic agents were developed by inhibiting a specific target, such as transcriptional activity, post-translation enzymatic steps, autophagy, and oxidative stress. These agents were tested in experimental models of cells of progeria yielding a modest improvements in phenotypes and slight extension of the lifespan of progeria patients. Since oxidative stress and chronic inflammation are involved in pathogenesis of progeria, this review proposes that a combination of a mixture of micronutrients with currently used therapeutic approaches may extend the life-span more than that produced by drug therapy alone.
Keywords:
Progeria; Mutation in LMNA gene; Accelerated aging; oxidative stress; Micronutrient mixture
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