About My Health Careers Internship MedBlogs Contact us
Medindia LOGIN REGISTER
Advertisement

Can Cholesterol-lowering Statins Ease Deadly Heart Condition in Rare Neuromuscular Disease?

by Thilaka Ravi on April 18, 2018 at 6:42 PM
Font : A-A+

Can Cholesterol-lowering Statins Ease Deadly Heart Condition in Rare Neuromuscular Disease?

In cell models that mimicked liver cells of patients with the rare disease Friedreich's ataxia (FA), a widely used cholesterol-lowering drug was found to increase a precursor of HDL (high-density lipoprotein), the 'good cholesterol,' in preclinical studies. The research is published in PLOS ONE from the Perelman School of Medicine at the University of Pennsylvania.

Decreased HDL and apolipoprotein A-I (ApoA-l) levels in the general population are associated with an increased risk of death from cardiomyopathy and heart failure. "We found the FA patients had serum ApoA-I levels that were lower than healthy control subjects," said senior author Ian Blair, PhD, a professor of Systems Pharmacology and Translational Therapeutics. The next step of the research, conducted in collaboration with David Lynch, MD, PhD, a professor of Neurology at Children's Hospital of Philadelphia (CHOP), showed that statins may be able to help Friedreich's ataxia patients increase their naturally low ApoA-I levels and so increase their HDL levels, which might help prevent heart disease.

Advertisement


Friedreich's ataxia (FA) is a rare condition diagnosed in about 50,000 people worldwide. It is the most common form of inherited ataxia, a condition characterized by a progressive loss of coordinated movement and balance. Onset of symptoms usually occurs in childhood, and most patients are confined to a wheelchair by their mid-to-late twenties. Most FA patients die from the thickening of their heart walls (hypertrophic cardiomyopathy) rather than from neurological issues.

In FA, the protein frataxin is made in insufficient amounts for mitochondria to function properly. This happens because of a mutation in the FXN gene, which results in reduced expression of frataxin protein. Frataxin is normally found in the mitochondria of cells and is involved in biochemical reactions that produce energy.
Advertisement

Using a highly specific and sensitive assay, the researchers found a 22 percent decrease in ApoA-I in the blood of FA patients compared with blood samples from non-affected healthy individuals. Using an experimental FA cell line, they also found that cells without frataxin produced lower levels of ApoA-I compared to controls. However, the cholesterol-lowering drug simvastatin increased the ApoA-I levels by 20 percent in these cells.

"I think that our findings provide compelling evidence for a trial of statins in FA patients to potentially reduce their cardiovascular risk," Blair said. One issue is that higher doses of statins are associated with myositis (muscle inflammation) and rhabdomyolysis (destruction of skeletal muscle) so it would be essential to carefully monitor these side effects, he noted. In addition, co-administration of coenzyme Q10 supplement could help reduce the risk of muscle-related side effects. Overall, Blair maintains that the potential benefits to FA patients with low ApoA-I levels taking low doses of statins outweigh the potential risks.

Source: Eurekalert
Advertisement

Advertisement
News A-Z
A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
News Category
What's New on Medindia
Printed Temperature Sensors help with Continuous Temperature Monitoring
Health Benefits of Giloy
Breast Cancer Awareness Month 2021 - It's time to RISE
View all

Medindia Newsletters Subscribe to our Free Newsletters!
Terms & Conditions and Privacy Policy.

More News on:
Cholesterol Cholesterol - The Enigma Chemical Heart Healthy Heart Statins Mitral Valve Prolapse Aortic Valve Stenosis Pericarditis 

Recommended Reading
Coronary Heart Disease
In coronary heart disease, blood is unable to flow through blocked arteries. The main symptom is ......
Lifestyle Paths to Prevent Heart Disease
Heart disease can be of many types depending upon whether they involve the heart muscles or artery ....
Congestive Heart Failure
Congestive heart failure (CHF) is a condition in which the heart fails to work adequately as a pump ...
CoQ10
Vitamin-like CoQ10 helps generate energy for the cells to function at their optimum level. ......
Aortic Valve Stenosis
Aortic valve Stenosis is an abnormal narrowing of the c valve. Symptoms include angina, and that of ...
Cholesterol
Cholesterol is produced by the body (liver) and is essential for normal body functioning....
Cholesterol - The Enigma Chemical
The word 'Cholesterol' rings danger bells as soon as it is mentioned. This important chemical is im...
Mitral Valve Prolapse
Mitral Valve Prolapse is a relatively common condition and causes leakage of blood through the valve...
Pericarditis
Pericarditis occurs when the pericardium gets inflamed. Pericarditis is characterized by severe ches...
Statins
Statins are new wonder drugs that are proving to be efficacious, not merely in relieving symptoms bu...

Disclaimer - All information and content on this site are for information and educational purposes only. The information should not be used for either diagnosis or treatment or both for any health related problem or disease. Always seek the advice of a qualified physician for medical diagnosis and treatment. Full Disclaimer

© All Rights Reserved 1997 - 2021

This site uses cookies to deliver our services. By using our site, you acknowledge that you have read and understand our Cookie Policy, Privacy Policy, and our Terms of Use