Hutchinson Gilford Progeria News and Research

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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

Scientists develop novel genetic model of premature aging disorders

Working with a group of national and international researchers, scientists from the Florida campus of The Scripps Research Institute have developed a new genetic model of premature aging disorders that could shed light on these rare conditions in humans and provide a novel platform for large-scale screening of compounds to combat these and other age-related diseases.

29 Apr 2011

Progeria-derived iPS cells may treat aging-related disease in children

The current pace of population aging is without parallel in human history but surprisingly little is known about the human aging process, because lifespans of eight decades or more make it difficult to study. Now, researchers at the Salk Institute for Biological Studies have replicated premature aging in the lab, allowing them to study aging-related disease in a dish.

24 Feb 2011

Human model of rare genetic disease reveals new clues to aging process

Scientists from A*STAR's Institute of Medical Biology (IMB) in Singapore and the University of Hong Kong's Department of Medicine have produced the world's first human cell model of progeria, a disease resulting in severe premature ageing in one in four to eight million children worldwide. This model has allowed them to make new discoveries concerning the mechanism by which progeria works.

25 Jan 2011

Progerin may contribute to risk of atherosclerosis: Study

Progeria, also known as Hutchinson-Gilford Progeria Syndrome (HGPS), is a rare, fatal genetic disease characterized by an appearance of accelerated aging in children. All children with Progeria die of the same heart disease that affects millions of normal aging adults (atherosclerosis), but instead of occurring at 60 or 70 years of age, these children may suffer heart attacks and strokes even before age 10, and the average age of death is 13 years.

8 Sep 2010

Secretary Sebelius announces Senate confirmation of Dr. Francis Collins as Director of NIH

HHS Secretary Kathleen Sebelius announced today that the United States Senate unanimously confirmed Dr. Francis Collins as the next Director of the National Institutes of Health.

10 Aug 2009

Promising clinical trial for Progeria "premature aging" disease

One of the fastest translations of a basic research discovery into a promising clinical trial for an "untreatable" and fatal disorder will be discussed publicly for the first time by the key players in this remarkable research story, on Sunday, Dec. 14, at the American Society for Cell Biology (ASCB)'s annual meeting in San Francisco.

14 Dec 2008

Adult stem cell changes underlie Hutchinson-Gilford Progeria syndrome associated with accelerated aging

Adult stem cells may provide an explanation for the cause of a Hutchinson-Gilford Progeria Syndrome (HGPS), a rare disease that causes premature aging in children, according to researchers at the National Cancer Institute (NCI), part of the National Institutes of Health (NIH).

3 Mar 2008

Researchers discover new cell structures

New breakthrough for children suffering from an extremely rare disease that accelerates the aging process by about seven times the normal rate.

28 Jun 2006

Farnesyltransferase inhibitor may improve progeria

UCLA researchers found that an experimental cancer drug improves the signs of progeria in a mouse model. Progeria is a rare genetic disease causing accelerated aging and cardiovascular disease in children. The new UCLA findings help to define a new strategy for treating children with progeria.

17 Feb 2006

Farnesyl transferase inhibitors may help chidren with Hutchinson-Gilford progeria syndrome

Johns Hopkins scientists have discovered that a drug currently being tested against cancers might help children with a rare, fatal condition called Hutchinson-Gilford progeria syndrome, which causes rapid, premature aging.

27 Sep 2005

Hutchinson-Gilford progeria syndrome in children may be helped by cancer drugs

Children who suffer from a rare disease called progeria, which accelerates aging and often kills patients when they are in their teens, may possibly be helped by drugs being developed to treat cancer.

31 Aug 2005