Link Between Calcium and Cardiolipin in Found Heart Deficiencies-Health

The heart needs energy to pump blood. Thus, defective myocardial energy production causes various heart diseases. In light of the information below, scientist A new link has been discovered between calcium, cardiac energy production, and a type of fat, cardiolipin.

This finding helps explain the rare genetic disorder Barth’s Syndrome cardiac defect.

of InvestigationPublished in the minutes of the National Academy of Sciences, led by Dr. Vishal M. Gohil of the Department of Biochemistry and Biophysics at the Texas A & M College of Life and Life Sciences. Other co-authors were from the University of Texas Health Science Center in San Antonio and the Massachusetts General Hospital in Boston. Funding for this study came from the Welch Foundation and the National Institutes of Health.

Barth Syndrome Heart Defect

Barth syndrome is a rare genetic disorder that affects only boys. Affected children have suffered heart and muscle weakness since infancy. With this debilitating illness, patients struggle with daily activities such as walking and running. Often, their hearts are weak and expanding.

People with Barth syndrome have a genetic defect that interferes with the body’s ability to make cardiolipin. As its name suggests, cardiolipin is found in large amounts in the heart-heart-muscle. Cardiolipin belongs to a class of molecules called lipids.

Cardiolipin is found in mitochondria within muscle cells. Mitochondria are known as “power plants” for cells because they produce biological energy from the food we eat. Cardiolipin and other lipids from the mitochondrial membrane “skin”, but cardiolipin appears to be a particularly important component. Lack of cardiolipin impairs mitochondria’s ability to generate energy in the form of adenosine triphosphate, ATP.

The link between cardiolipin, energy and calcium

When cells need a burst of energy, they use calcium as a signal to encourage mitochondria to increase energy production. Calcium ions enter mitochondria through special channels in the mitochondrial membrane. Since calcium channels reside in the same membrane as cardiolipin and other lipids, Gohil and his team wondered how lipids affect channels.

“I knew that this channel was in the mitochondrial membrane, so I asked if the lipids in the membrane could affect the function of this channel,” Gohil said.

Baker’s yeast supports research on energy production in Barth syndrome

Gohill’s lab had previously found a way to make yeast mitochondria lacking various lipids, including cardiolipin. Yeast have mitochondria, which in many respects mimic human mitochondria, but lack calcium channels.

The lead author of the study, a graduate student at Gohil’s lab, Sagnika Ghosh genetically modified the mitochondria of transgenic baker’s yeast to include human calcium channels. Next, we investigated what happens to calcium transport when the lipid composition of the membrane changes.

“We found that calcium syndrome was not stable in mitochondrial membranes, which are low in cardiolipin, such as those found in patients with Baltic syndrome,” Gohil said.

Confirm patient sample

Next, the team obtained cell and heart tissue samples from patients with Barth syndrome. The team confirmed that what they saw in the yeast experiment was also occurring in the patient sample. Due to the instability of mitochondrial calcium channels, mitochondria in patients with Barth syndrome were much less permeable to calcium than in healthy cells.

Therefore, if the patient’s cells require a burst of energy, sending a calcium signal to the mitochondria may not increase energy production as it would in healthy cells.

“Starting with basic scientific questions, our research has led to discoveries related to human health,” Gohhill said. “In this genetic disorder, defective calcium uptake can affect energy production. What we observed in yeast also applies to human cells.”

(This article has been published from the telegraph agency feed with no changes to the text. Only the headings have changed.)

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