Hutchinson Gilford Progeria News and Research

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New progeria-on-a-chip model provides better understanding of vascular disease and aging

Hutchinson-Gilford progeria syndrome is an extremely rare genetic condition that causes premature and accelerated aging.

3 Mar 2017

Researchers identify heart defects in progeria patients that may raise risk of arrhythmias, premature death

Researchers at the Centro Nacional de Investigaciones Cardiovasculares Carlos III, working in collaboration with colleagues at other centers in Spain and abroad, have identified defects in the hearts of progeria patients that appear to be related to an elevated risk of arrhythmias and premature death.

2 Nov 2016

Embryonic stem cell gene Nanog holds potential for reversing effects of aging

The fountain of youth may reside in an embryonic stem cell gene named Nanog.

26 Jul 2016

ANK3 and other genes may play key role in affecting mood, stress and longevity

The visible impacts of depression and stress that can be seen in a person's face -- and contribute to shorter lives -- can also be found in alterations in genetic activity, according to newly published research.

24 May 2016

Study finds link between PTSD and accelerated aging

In recent years, public health concerns about post-traumatic stress disorder (PTSD) have risen significantly, driven in part by affected military veterans returning from conflicts in the Middle East and elsewhere. PTSD is associated with number of psychological maladies, among them chronic depression, anger, insomnia, eating disorders and substance abuse.

10 May 2015

Study provides common model of cellular ageing

Researchers at the BBSRC-supported Babraham Institute have mapped the physical structure of the nuclear landscape in unprecedented detail to understand changes in genomic interactions occurring in cell senescence and ageing. Their findings have allowed them to reconcile the contradictory observations of two current models of ageing: cellular senescence of connective tissue cells called fibroblasts and cellular models of an accelerated ageing syndrome.

2 Feb 2015

Hutchinson-Gilford Progeria Syndrome can be compared to normal aging, say scientists

In a new research study, scientists from Vision Genomics, LLC, Insilico Medicine, Inc., and Howard University showed that Hutchinson-Gilford Progeria Syndrome (HGPS or Progeria) is comparable to normal aging with respect to cellular signaling pathways, and that HGPS truly recapitulates the normal aging process.

28 Jan 2015

Broccoli can help reduce HGPS-related defects

Children who suffer from Hutchinson-Gilford Progeria syndrome age prematurely due to a defective protein in their cells. Scientists at Technische Universität München have now identified another important pathological factor: the system responsible for removing cellular debris and for breaking down defective proteins operates at lower levels in HGPS cells than in normal cells. The researchers have succeeded in reactivating protein breakdown in HGPS cells and thus reducing disease-related defects by using a substance from broccoli.

18 Dec 2014

Researchers discover role played by epigenetic factors in senescence and age-related diseases

One way cells promote tumor suppression is through a process called senescence, an irreversible arrest of proliferation. Senescence is thought to be associated with normal aging, but is also a protective measure by the body against run-away cell replication.

31 Aug 2013

New study identifies possible treatment to block deposition of calcium in arterial wall

HGPS is a rare genetic disease that affects one in every 4-8 million births. The disease is caused by a spontaneous mutation in one of the two copies (alleles) of the gene LMNA, which codes for lamin A, a protein important for the integrity and function of the envelope surrounding the cell nucleus. The mutation causes incorrect processing of the messenger RNA for lamin proteins, resulting in the synthesis of an anomalous protein, called progerin.

18 Jun 2013

New breakthrough in progeria treatment

Pharmaceuticals that inhibit a specific enzyme may be useful in treating progeria, or accelerated aging in children. A new study performed at the Sahlgrenska Academy, University of Gothenburg, Sweden, indicates that the development of progeria in mice was inhibited upon reducing the production of this enzyme.

21 May 2013

Mutant protein contributes to Hutchinson-Gilford Progeria syndrome

A mutant protein responsible for Hutchinson-Gilford Progeria syndrome (HGPS) bars large proteins from entering the nucleus, according to a study in The Journal of Cell Biology.

7 May 2013

Evidence shows progerin induced by ultraviolet light is responsible for premature skin aging

A study conducted by researchers at Boston University School of Medicine (BUSM) provides new evidence that longwave ultraviolet light (UVA) induces a protein that could result in premature skin aging.

26 Apr 2013

Scientists discover new evidence for role of epigenetic changes on premature aging diseases

Scientists at the Bellvitge Biomedical Research Institute in Barcelona, Spain have found new evidence for the role of epigenetic changes (those heritable alterations in gene expression caused by mechanisms other than changes in DNA sequence) on premature aging diseases.

1 Feb 2013

Lonafarnib clinical trial for progeria has scientific roots in basic biology discoveries

The good news widely reported this morning of positive results from a clinical drug trial at Boston Children's Hospital for the previously "untreatable" rapid aging disorder in children known as progeria has its scientific roots in basic biology discoveries made in recent years.

26 Sep 2012

Lonafarnib proves effective for children with progeria

Results of the first-ever clinical drug trial for children with Progeria, a rare, fatal "rapid-aging" disease, demonstrate the efficacy of a farnesyltransferase inhibitor (FTI), a drug originally developed to treat cancer. The clinical trial results, completed only six years after scientists identified the cause of Progeria, included significant improvements in weight gain, bone structure and, most importantly, the cardiovascular system, according to The Progeria Research Foundation (PRF) and Boston Children's Hospital.

25 Sep 2012

Rapamycin improves function and extends survival in mice with DCM and rare muscular dystrophies

Rapamycin, an immunosuppressant drug used in a variety of disease indications and under study in aging research labs around the world, improved function and extended survival in mice suffering from a genetic mutation which leads to dilated cardiomyopathy (DCM) and rare muscular dystrophies in humans.

26 Jul 2012

Potential drug therapy for Hutchinson Gilford Progeria Syndrome

Researchers have identified a potential drug therapy for a premature ageing disease that affects children causing them to age up to eight times as fast as the usual rate.

2 Nov 2011

Pill for rare ageing disease on the horizon

Scientists have found that a drug used in transplant patients could reverse the effects of premature ageing and extend human life by over 10 years.

1 Jul 2011

Salk researchers successfully edit diseased gene in patient-specific induced pluripotent stem cells

In principle, genetic engineering is simple, but in practice, replacing a faulty gene with a healthy copy is anything but. Using mutated versions of the lamin A gene as an example to demonstrate the versatility of their virus-based approach, researchers at the Salk Institute for Biological Studies successfully edited a diseased gene in patient-specific induced pluripotent stem cells as well as adult stem cells.

21 May 2011