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Progeria-a Rare Genetic Condition with Accelerated Ageing Process.

Pratik Talukder1, Arunima Saha2, Sohini Roy2

  • 1Department of Biotechnology, University of Engineering and Management, University Area, Plot, Street Number 03, Action Area III, B/5, Newtown, Kolkata, West Bengal, 700156, India. pratik.talukder@uem.edu.in.

Applied Biochemistry and Biotechnology
|April 21, 2022
PubMed
Summary

Progeria, a rare genetic condition causing rapid aging, significantly shortens lifespan. Current research explores genetic causes and therapeutic strategies for Hutchinson-Gilford progeria syndrome (HGPS), though effective treatments remain limited.

Keywords:
Clinical trialsExperimental modelsHutchinson-Gilford progeria syndrome (HGPS)Lamin ALonafarnibProgerinTranslational

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Area of Science:

  • Genetics
  • Molecular Biology
  • Pediatrics

Background:

  • Progeria is a rare genetic disorder characterized by accelerated aging and a significantly reduced lifespan.
  • Hutchinson-Gilford progeria syndrome (HGPS) is the classic form, presenting symptoms like low weight, scleroderma, osteoporosis, and hair loss within the first year of life.
  • The average life expectancy for individuals with HGPS is approximately 14.6 years.

Purpose of the Study:

  • To review recent advancements in understanding the genetic and molecular basis of progeria.
  • To explore current and emerging therapeutic approaches for progeria.
  • To provide a comprehensive overview of progeria's epidemiology, symptoms, diagnosis, and treatment options.

Main Methods:

  • Literature review of experimental models, drugs, and molecular technologies.
  • Analysis of recent developments in progeria research.
  • Synthesis of information on epidemiology, genetics, symptoms, diagnosis, and treatment.

Main Results:

  • Research is ongoing to elucidate the genetic and molecular causes of progeria.
  • Therapeutic strategies and drug development for progeria show limited success rates currently.
  • Various experimental models, drugs, and molecular technologies are under investigation to improve understanding and treatment.

Conclusions:

  • A deeper understanding of progeria requires further investigation into its genetic and molecular underpinnings.
  • Developing effective treatments for progeria remains a significant challenge.
  • Continued research into experimental models and novel therapies is crucial for improving outcomes for individuals with progeria.