“This study is a breakthrough for our research group after years of work.”
When we reduce the production of the enzyme in mice, the development of all the clinical symptoms of progeria is reduced or blocked. We have also studied cultured cells from children with progeria, and can see that when the enzyme is inhibited, the growth of the cells increases by the same mechanism as in mouse cells,” says Martin Bergö, Professor at the Sahlgrenska Academy, University of Gothenburg and research director at the Sahlgrenska Cancer Center.
Progeria is a rare genetic childhood disorder characterized by the appearance of accelerated aging. The classical form of progeria, called Hutchinson-Gilford Progeria Syndrome (HGPS), is caused by a spontaneous mutation, which means that it is not inherited from the parents.
Children with HGPS usually die in their teenage years from myocardial infarction and stroke.
The progeria mutation occurs in the protein prelamin A and causes it to accumulate in an inappropriate form in the membrane surrounding the nucleus. The target enzyme, called ICMT, attaches a small chemical group to one end of prelamin A. Blocking ICMT, therefore, prevents the attachment of the chemical group to prelamin A and significantly reduced the ability of the mutant protein to induce progeria.
“We are collaborating with a group in Singapore that has developed candidate ICMT inhibitor drugs and we will now test them on mice with progeria. Because the drugs have not yet been tested in humans, it will be a few years before we know whether these drugs will be appropriate for the treatment of progeria,” Martin Bergö explains.
The Latest Bing News on:
- Many parents of kids with rare diseases fight for research — even if it’s too late for their childon February 27, 2024 at 1:20 pm
"We’re still participating in this research even though our son passed away, and that still adds meaning to his life." ...
- Turning to Patients to Help Drive Rare Disease Drug Developmenton February 25, 2024 at 9:33 pm
Patient advocacy groups aided in the development of the very first marketed drugs for certain rare diseases, including progeria and Friedreich’s ataxia.
- New study aims to define the progeria phenomeon February 21, 2024 at 9:28 am
Progeroid disorders are a heterogenous group of rare and complex hereditary syndromes presenting with pleiotropic phenotypes associated with normal aging. Due to the large variation in clinical ...
- New study: Defining the progeria phenomeon February 20, 2024 at 2:56 pm
“In sum, we have defined what a premature aging disease is and developed tools to allow diagnostics of patients and disease population.” “In this study, we have utilized phenome explorations ...
- Base Editing in Progeriaon January 24, 2024 at 4:00 pm
This is also a question that scientists ask themselves when trying to find a truly effective treatment for patients with the Hutchinson–Gilford progeria syndrome (HGPS) and other genetic diseases.
- YouTuber Adalia Rose dies aged 15 from genetic conditionon December 29, 2023 at 10:24 am
The US teenager was diagnosed with Hutchinson-Gilford progeria, also known as Benjamin Button disease, when she was three months old. It's a rare fatal condition which causes problems with growth ...
- Phenotype and Course of Hutchinson–Gilford Progeria Syndromeon November 8, 2023 at 1:21 pm
Hutchinson–Gilford progeria syndrome is a rare, sporadic, autosomal dominant syndrome that involves premature aging, generally leading to death at approximately 13 years of age due to myocardial ...
- Causeson November 6, 2023 at 5:38 am
For informational purposes only. Consult a medical professional for advice. Source: Focus Medica. A progressive genetic disorder where symptoms of aging are manifested at an early age. This ...
- Werner and Hutchinson–Gilford progeria syndromes: mechanistic basis of human progeroid diseaseson July 7, 2023 at 2:34 pm
Two of the best characterized progeroid syndromes are Werner syndrome and Hutchinson–Gilford progeria syndrome (HGPS). The genes targeted for mutation in these diseases (WRN and LMNA ...
- Progeria syndromes and ageing: what is the connection?on October 31, 2022 at 2:10 am
Hutchinson–Gilford progeria syndrome (HGPS) and other human progerias allow researchers a unique glimpse at the molecular pathways that accelerate age-associated phenotypes. These insights can ...
The Latest Google Headlines on:
[google_news title=”” keyword=”progeria” num_posts=”10″ blurb_length=”0″ show_thumb=”left”]
The Latest Bing News on:
- 5 Skin Hydrating Supplements To Support Healthy Aging — And Your Glowon February 29, 2024 at 3:05 am
while helping combat accelerated aging and improving skin clarity. Add a heaping teaspoon to your warm matcha, lemonade or any smoothie. Zinc and Selenium in Dr. Barbara Sturm's anti-aging suppelment ...
- Scientists find leaky gut may be linked to faster biological agingon February 28, 2024 at 7:45 pm
Recent research published in the journal Microbiome revealed a link between viral damage to the gut and accelerated biological aging. The findings suggest that this association can lead ...
- Wistar Scientists Discover Link Between Leaky Gut and Accelerated Biological Agingon February 23, 2024 at 8:12 am
Dr. Mohamed Abdel-Mohsen and collaborators discovered a connection between gut damage and premature biological aging in people living with chronic viral infectionPHILADELPHIA, PA, Feb. 23, 2024 (GLOBE ...
- Accelerated Epigenetic Aging Seen in Women With HIVon February 22, 2024 at 10:30 pm
Accelerated epigenetic aging is seen in women with HIV versus women without HIV, according to a study published online Feb. 15 in the Journal of Infectious Diseases. Stephanie Shiau, Ph.D., from the ...
- Our aging candidates spark a valuable discussion on brain healthon February 22, 2024 at 9:00 am
After the acute infection, many patients complain of brain fog, memory and attention lapses, challenges with finding words and multi-tasking and even personality changes. Some recover over time from ...
- Leaky Gut and Accelerated Biological Aging Linkedon February 22, 2024 at 1:11 am
Viral damage to the gut has been linked to premature biological aging through the impact of leaky gut on the immune system.
- First-of-its-Kind Study Reveals Accelerated Aging in Women with HIVon February 16, 2024 at 9:48 am
Managed Healthcare Executive provides C-suite executives at health plans and provider organizations with news, analysis, insights, and strategies to pursue value-driven solutions.
- Gender variations in brain aging among Parkinson's Disease patientson February 16, 2024 at 6:30 am
In a recent study published in npj Parkinson’s Disease, researchers explored how sex influences brain aging in Parkinson's disease (PD) by analyzing Magnetic Resonance Imaging (MRI)-derived brain age ...
- Women with HIV experience accelerated DNA aging, study showson February 15, 2024 at 10:44 am
Women with HIV experience accelerated DNA aging, a phenomenon that can lead to poor physical function, according to a study led by Stephanie Shiau, an assistant professor in the Department of ...
The Latest Google Headlines on:
[google_news title=”” keyword=”accelerated aging” num_posts=”10″ blurb_length=”0″ show_thumb=”left”]