Navigation Links
Adult stem cell changes underlie rare genetic disease 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). These findings, the first to indicate a biological basis for the clinical features of HGPS, also known as progeria, may also provide new insights into the biological mechanisms of normal aging. The results were published in the March, 2008, issue of Nature Cell Biology.

Studies like this of the biology of HGPS hold the potential to benefit children suffering this terrible illness and enlighten us as to the medical changes we all experience as we grow older. said NCI Director John E. Niederhuber, M.D. As our population ages, we have an increasing need for greater understanding of the biology of aging and age-related illness, such as cancer.

HGPS is an extremely rare hereditary genetic disease of children characterized by signs of premature aging. Children with HGPS generally experience the first symptoms by the age of one, and on average succumb around the age of 15, almost exclusively from premature, progressive heart disease. HGPS occurs in one out of four to eight million births; only 100 patients have been documented in the medical literature. Because its striking cardiovascular effects and other clinical features are so closely associated with the normal aging process, HGPS holds great interest for researchers studying age-related biological changes and disease.

The cause of HGPS, a mutated protein called progerin, was identified in 2003. However, the mechanism by which progerin causes the widespread clinical effects of HGPS has been unclear. To forge this link between molecular biology and medical outcome, Tom Misteli, Ph.D., head of the Cell Biology of Genomes Group at NCIs Center for Cancer Research (CCR), and CCR staff scientist Paola Scaffidi, Ph.D., examined the effects of progerin on gene expression in a laboratory model of HGPS. They found that progerin activates genes involved in the Notch signaling pathway, a major regulator of stem cell differentiation -- the process by which stem cells give rise to the mature cells that make up different tissues.

Because most of the tissues affected by HGPS (e.g., skin, fat, muscles, bone, and blood vessels) arise from a common developmental pathway, Misteli and Scaffidi looked at the effects of progerin on adult mesenchymal stem cells, the common cellular ancestor of these tissue types. An adult stem can renew itself, and can differentiate to yield the major specialized cell types of the tissue or organ. Their experiments revealed that progerin profoundly affects the fate of these stem cells, greatly skewing the rate at which they mature into different tissues. For instance, progerin-producing stem cells showed accelerated maturation into bone but failed to develop into fat. This could explain two of the distinguishing clinical features of HGPS: abnormal bone growth and an almost complete loss of the fatty tissues normally found just beneath the skin. The researchers were able to mimic the progerins effects in these stem cells by experimentally activating the same components of the Notch pathway targeted by progerin.

Taken together, the results of these experiments provide a new window into the biology behind the clinical features of HGPS. They may also hold relevance for understanding the biology of normal aging. Progerin is present at low levels in the cells of healthy people, said Misteli. One could envision a scenario in which progerins effects on the Notch pathway and, by extension, on adult stem cells could, over time, lead to many of the tissue changes we commonly associate with the aging process.


Contact: NCI Press Officers
NIH/National Cancer Institute

Related medicine news :

1. YMCA of the USA and McNeil Consumer Healthcare Partner to Promote Health for Older Adults
2. Adult Stem Cells Help Those With Immune Disorders, Heart Disease
3. A rare diagnosis in the operation room: Kidney atrophy due to duplicated colon in an adult
4. Cognitive Impairment Declining in Older Adults
5. CME LLC Offers Education to Help Medical Professionals Better Recognize Adult ADHD
6. Hand-held computers prod older adults to exercise more, Stanford study shows
7. JDRF partners with Plureon to explore generating insulin-producing cells from adult stem cells
8. Modified Atkins diet can cut epileptic seizures in adults
9. Anti-Inflammatory Drug Improves Glycemia, Lowers Inflammation in Obese, Young Adults
10. Teens, Young Adults Missing Out on Latest Cancer Advances
11. Turning on adult stem cells may help repair bone
Post Your Comments:
(Date:10/12/2017)... Fla. (PRWEB) , ... October 12, 2017 , ... IsoComforter, ... therapy products, announced today the introduction of an innovative new design of the shoulder ... pad so you get maximum comfort while controlling your pain while using cold therapy. ...
(Date:10/12/2017)... ... ... Asante, a nationally recognized health system in southern Oregon, ... health joint venture through an agreement, effective October 1, 2017, to create AccentCare ... company with Asante, delivering clinically integrated care, for the past eight years. This ...
(Date:10/12/2017)... ... October 12, 2017 , ... ... Restoration, has recently contributed a medical article to the newly revamped Cosmetic ... Mohebi’s article spotlights the hair transplant procedure known as Follicular Unit Extraction ...
(Date:10/12/2017)... ... ... Leading pediatric oncology experts at Children’s National Health System will join ... International Society of Paediatric Oncology (SIOP) Oct. 12-15. Chaired by Jeffrey Dome, ... at Children’s National, and Stephen P. Hunger, M.D., Chief of the Division of ...
(Date:10/12/2017)... ... October 12, 2017 , ... Women-owned and Grand ... Best and Brightest in Wellness® by Best and Brightest. OnSite Wellness will be ... Oct. 20 from 7:30 a.m. to 2 p.m. at the Henry Autograph Collection ...
Breaking Medicine News(10 mins):
(Date:10/2/2017)... 2017  AllianceRx Walgreens Prime, the combined central specialty ... pharmacy benefit manager Prime Therapeutics LLC (Prime), today officially ... the unveiling of new signage at its headquarters in ... at a few other company-owned facilities across the country. ... some of whom will begin to see the AllianceRx ...
(Date:9/27/2017)... , Sept. 27, 2017  Commended for their devotion to ... awards. Ranked as number one in the South Florida Business ... in Inc. 5000 yearly list, the national specialty pharmacy has ... Armando Bardisa will soon be honored by SFBJ as ... Set to receive his award in October, ...
(Date:9/22/2017)... , Sept. 22, 2017  As the latest ... Senators Bill Cassidy (R-LA) and Lindsey ... notes that the medical device industry is in an ... device tax, the 2.3% excise tax on medical device ... they also want covered patients, increased visits and hospital ...
Breaking Medicine Technology: