A therapeutic goal It is a substance that is found in every part of a cell and is capable of recognizing a drug and causing a cellular response. A group from the National Center for Cardiovascular Research (CNIC) made the first discovery protects the heart in patients with pulmonary hypertension.
The work was published in the magazine today Scientific advancesrepresents a further step in the fight against this rare but fatal disease for which there is no cure. It is characterized by high pressure in the arteries leading to the lungs, which leads to constant overstrain of the heart.
To date, existing therapies target the lungs to lower blood pressure. However, these techniques still do not improve heart function Heart failure is the leading cause of death.
The team led by Guadalupe Sabio (currently at the National Cancer Research Center, CNIO), discovered in animal models using mice and pigs that patients with chronic obstructive pulmonary disease or COPD They had higher values mitochondrial protein called MCJ.
“COPD is a disease that causes fibrosis in the lungs. Nevertheless, the patient dies not from a lung problem, but from a specific heart failure, because the heart does not adapt well to this lack of oxygen,” Sabio tells SINC. “And if we can adapt the organ well to hypoxia, it would be possible to protect it.”
After confirming what happens in the hearts of pigs in humans, the researcher explains how the mice in which the MCJ protein was eliminated were completely protected from this lack of oxygen and did not develop heart failure in the right ventricle.
“If you removed it from all organs, they wouldn't even develop lung damage, but if you removed it only from the heart, we could protect it from hypoxia,” he points out. In short, we can protect ourselves from disease simply by changing metabolism in the heart.”

Ayelén M. Santamans and Guadalupe Sabio, first author and principal investigator of the study. / CNIC
New therapies against the disease
The new work shows how modulating levels of the mitochondrial protein MCJ in the heart can preserve cardiac function despite lung damage. “This protection comes from activating a signaling pathway that is essential for adapting to low oxygen levels and preparing the heart to function properly even in the absence of oxygen,” he emphasizes. Ayelén M. SantamansFirst author of the article.
The authors conclude that these findings may open new doors for possible therapeutic interventions against pulmonary hypertension. “The results suggested that the MCJ protein may be involved in the disease, and the lack of specific therapies motivated us to move forward in this direction,” he says.
Poor quality of life for patients
This disease affects between 15 and 50 people per million inhabitants. In Spain, a prevalence of 1.6 cases and an incidence (newly diagnosed cases per year) of 0.3 per 100,000 inhabitants are calculated.
Although it is a rare disease, those affected usually suffer from symptoms that significantly affect their quality of life: difficulty breathing, dizziness and fainting. In the most severe cases, a transplant is even necessary.
There are several risk factors that contribute to its development, such as: B. Smoking, obesity, previous illnesses, genetic predisposition or low oxygen levels in high-altitude areas.
Reference:
Ayelén M. Santamans et al.: “MCJ: A mitochondrial target for cardiac interventions in pulmonary hypertension.” Scientific advances 2024.
This study was funded by the Ministry of Science and Innovation, the IMPACT 2021 project, the Jesús Serra Foundation, the EFSD/Lilly European Diabetes Research Program, the BBVA Foundation, the Community of Madrid and the AECC.