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Discordant Gene Expression Signatures and Related Phenotypic Differences in Lamin A- and A/C-Related Hutchinson-Gilford Progeria Syndrome (HGPS) [PDF]

PLoS ONE, 2011
Hutchinson-Gilford progeria syndrome (HGPS) is a genetic disorder displaying features reminiscent of premature senescence caused by germline mutations in the LMNA gene encoding lamin A and C, essential components of the nuclear lamina.
Karl Heinimann, Gabor Szinnai, Philippe Demougin   +2 more
exaly   +3 more sources

Pluripotent stem cells to model Hutchinson-Gilford progeria syndrome (HGPS): Current trends and future perspectives for drug discovery [PDF]

Ageing Research Reviews, 2015
Progeria, or Hutchinson-Gilford progeria syndrome (HGPS), is a rare, fatal genetic disease characterized by an appearance of accelerated aging in children.
Xavier Nissan
exaly   +3 more sources

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Hutchinson-Gilford Progeria Syndrome (Hgps) and Application of Gene Therapy Based Crispr/Cas Technology as A Promising Innovative Treatment Approach

Recent Patents on Biotechnology, 2021
BACKGROUND Hutchinson-Gilford progeria syndrome (HGPS) also known as progeria of childhood or progeria is a rare, rapid, autosomal dominant genetic disorder characterized by premature aging which occurs shortly after birth.
Meenu Vijayan, Chameli Ratan, Mekha Rajeev   +2 more
exaly   +2 more sources

Altered modulation of lamin A/C‐HDAC2 interaction and p21 expression during oxidative stress response in HGPS

Aging Cell, 2018
Defects in stress response are main determinants of cellular senescence and organism aging. In fibroblasts from patients affected by Hutchinson–Gilford progeria, a severe LMNA‐linked syndrome associated with bone resorption, cardiovascular disorders, and
Cecilia Garofalo, Katia Scotlandi, Antonello Mai   +2 more
exaly   +2 more sources

Towards delineating the chain of events that cause premature senescence in the accelerated aging syndrome Hutchinson–Gilford progeria (HGPS)

Biochemical Society Transactions, 2020
The metazoan nucleus is equipped with a meshwork of intermediate filament proteins called the A- and B-type lamins. Lamins lie beneath the inner nuclear membrane and serve as a nexus to maintain the architectural integrity of the nucleus, chromatin ...
Oliver Dreesen
exaly   +2 more sources

HGPS

2021
exaly   +2 more sources

HGPS Animal Models

2021
exaly   +2 more sources

HGPS Model Organisms

2021
exaly   +2 more sources

A homozygousZMPSTE24null mutation in combination with a heterozygous mutation in theLMNAgene causes Hutchinson-Gilford progeria syndrome (HGPS): insights into the pathophysiology of HGPS

Human Mutation, 2006
Hutchinson‐Gilford progeria syndrome (HGPS) is a rare premature aging disorder normally caused by a spontaneous heterozygous mutation in the LMNA gene that codes for the nuclear lamina protein lamin A.
J. Denecke, T. Brune, Tobias Feldhaus, H. Robenek, C. Kranz, R. Auchus, A. Agarwal, T. Marquardt   +7 more
exaly   +2 more sources

Pathological modelling of pigmentation disorders associated with Hutchinson-Gilford Progeria Syndrome (HGPS) revealed an impaired melanogenesis pathway in iPS-derived melanocytes [PDF]

Scientific Reports, 2018
International audienceHutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic disorder that leads to premature aging. In this study, we used induced pluripotent stem cells to investigate the hypopigmentation phenotypes observed in patients with ...
Nicolas Levy, Christine Baldeschi
exaly   +4 more sources