An Updated Review On Hutchinson-Gilford Progeria Syndrome

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DOI: 10.4103/2231-4040.197331

In 1886 Jonathan Hutchinson first identified the disease “Progeria” and thereafter by Hastings Gildford in 1897. It was later renamed as Hutchinson-Gilford Progeria syndrome (HGPS). HGPS is a genetic disorder identified by accelerated aging immediately after birth in the first year of life. Clinical trials by using different drugs either alone or in combination are under progress. Recently it was discovered that rapamycin is efficient in not only clearing defective protein progerin but also inhibits geroconversion process. Another drug resveratrol improved lifespan of patients. Accumulation of progerin activates NF-kB pathway which produces inflammatory cytokines leading to inflammation of progeroid mice, this pathway was inhibited by simultaneous use of statins, aminobisphosphonates and Non-Steroidal Anti-inflammatory Drugs (NSAIDs). Recently, a new technology named CRISPR/Cas system permits to edit genome at specific loci which is permanent. The combination of non-integrating transient vectors with CRISPR/Cas constructs proven effective for the treatment of HGPS. This review summarizes recent updates made in understanding of HGPS and focuses on recent developments in research of HGPS in terms of physiological processes and drug discovery.

Keywords: HGPS, Lamins, FTIs, Rapamycin, Resveratrol, Crispr Constructs