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A glimmer of hope: Researcher creates cell lines to help treat mitochondrial diseases in children

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An artistic rendering of a Mitochondrion inside of a human cell. Photo courtesy of Adobe Stock.

The mitochondrion has garnered quite the reputation for its role as the “powerhouse of the cell.” These tiny, but mighty organelles play various life-sustaining roles, from powering our own cells and organs to fuelling chemical and biological processes. But when they aren’t working properly, a number of rare diseases can occur.

Mitochondrial diseases are a group of debilitating genetic disorders that affect one in 5,000 people throughout the world, most of them being children. Along with these diseases come a variety of health concerns, including, but not limited to; heart disease, developmental and cognitive disabilities, respiratory issues, poor growth, and even premature death. As of this moment, there is no cure.

But recent work published in the journals Mitochondrion and BMC Molecular and Cell Biology by Aloka Abey Bandara, a research associate professor in the Department of Biomedical Sciences and Pathobiology in the Virginia-Maryland College of Veterinary Medicine, and his team offers mitochondrial disease patients and their parents a glimmer of hope.

Bandara has successfully created live cell models that mimic mitochondrial disease cells. These cells will lay the groundwork for drug studies and future studies for mitochondrial diseases.

“Our cell models will allow us to see what exactly happens to the cells and its processes when a child develops mitochondrial disease. In addition to these factors, we will be able to conduct further study into the toxicity and effectiveness of novel drug candidates,” said Bandara.

Our bodies produce life-sustaining energy from the food we eat and the air we breathe. Oxygen and nutrients, like glucose, travel throughout the body’s organs, tissues, and cells until they arrive at their final destination: the mitochondria. When the nutrients reach the inner membrane of the mitochondria, a unique series of protein complexes, called the electron transport chain, kicks into gear.

Through a series of reactions, the electron transport chain is able to remove electrons from the nutrients and push them through the mitochondrial membrane, which forms a gradient of protons. When this happens, the body generates adenosine triphosphate, better known as ATP, a molecule that carries energy within cells.

“Sometimes, you can see disruptions or mutations within the electron transport chain proteins,” said Bandara. “As a result, the protein complexes cannot transport electrons, and then energy production is disrupted. Almost all organs of the organism will be affected – heart, eyes, and muscles – and they will not be able to function correctly.”

The electron transport chain is composed of five protein complexes, or groups of proteins. Complex I and Complex II are two protein complexes that are primarily responsible for removing electrons from the nutrients. If they fail to do their duty, the whole electron transport chain fails, and the body cannot produce ATP.

Mitochondrial disease patients can have defects in either Complex I or Complex II. Patients who have Complex I disruptions typically have neurological problems such as seizures and abnormal brain functions. Those with Complex II disruptions can develop many other diseases and are more likely to develop several cancers.

Although researchers are able to pinpoint where exactly the defects are located, creating treatments for these mitochondrial diseases has been a challenge. Therapies, vitamins, and dietary adjustments have been able to help alleviate symptoms and slow down the progression of disease; but, mitochondrial disease, itself, does not have a cure. Therefore, new drugs need to be created, tested, and refined.

Bandara hopes that his cell lines will not only support upcoming research, but also the patients and their families, who are experiencing the mitochondrial disease and all of its impacts firsthand.

“The parents are often helpless because they cannot just go to the pharmacy and get a drug,” said Bandara. “Hopefully, they can see that researchers are taking huge leaps to find a cure for these diseases. Maybe they can feel that they are not alone anymore – that the universities, the government, and science are fighting with them.”

In order to test drug candidates, researchers must first create cellular models, which act as artificial “sick” cells. Cellular models are an excellent tool for drug discovery because mitochondrial disease can be studied without actually needing to extract cells from patients.

To create cells that mimic mitochondrial disease, Bandara had to “knock-out” portions of the genome that create the coding for Complex I and Complex II using CRISPR/Cas9 technology.

First, researchers identified the portion of the genome that needed to be deleted. Then, they designed a piece of RNA that made that point it’s “home base”. The RNA then “guided” an enzyme called Cas9 to its home base on the gene. Cas9 is then able to bind to that point and “cut” it.

After this process was completed, Bandara ran genomic sequencing to confirm that the portion was successfully deleted from the genome. Over several months of hard work, Bandara and his team created two mutant cell lines, one with Complex I removed, and the other without Complex II.

Bandara is one of the few researchers in the world to use the CRISPR/Cas9 technology.

After the mutant cell lines were created, Bandara ran them through a disease model, where he tested the functions of the “sick” cell line against the “parent” cell line, which is composed of healthy cells. Through close analysis, Bandara confirmed that the sick cells consumed far less oxygen, grew very slowly, and did not produce enough ATP for the cells to function properly – the three trademarks of cells with mitochondrial disease.

Once they confirmed that the knockout cell lines properly simulated the cellular dysfunctions of mitochondrial disease, they were able to test a newly developed drug called Idebenone. With this treatment, Bandara showed that cell growth and oxygen consumption can be restored to a certain extent.

These cell lines were the product of a fruitful collaboration of experts from the Virginia Tech Department of Human Nutrition, Foods, and Exercise and the Virginia Tech-Carilion School of Medicine. The construction of mutant cell lines was guided and supported by David Brown, a former associate professor in the Department of Human Nutrition, Foods, and Exercise in the Virginia Tech College of Agriculture and Life Sciences, now the Senior Director of Scientific and Technical Innovation at Stealth BioTherapeutics, a Boston-based biotechnology company.

From this work, the team has received two provisional patents for their cells. These cell lines have already been licensed to two pharmaceutical companies in Boston and Chicago, which will develop new therapies for individuals suffering from mitochondrial diseases.

The cells have been made available for global use by interested researchers and pharmaceutical companies through Ximbio, the world’s largest non-profit specializing in life science research tools of all types.

“Cell models of mitochondrial complex I and II defects carry high societal and economic impact as models for testing drug candidates in a cost- and time-effective manner for the treatment of mitochondrial dysfunction” said Dr. Justin Perry, the business development manager at Ximbio.



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From stabilisation to transformation without delay

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At a symposium on reconciliation organised by the National Peace Council last week, more than 250 religious clergy, civic activists and political representatives from different communities gathered to discuss the country’s future. Speaking at the event, Minister Bimal Rathnayake explained the government’s approach to national reconciliation. He said the government viewed the country’s recovery in terms of a three stage process. The first stage was stabilisation, the second was development and the third was transformation. Reconciliation, he implied, would come in that final stage. The participation of Opposition Leader Sajith Premadasa at the same symposium, and the constructive nature of his comments, strengthens that hope.

When the present NPP government took office in 2024, the country was emerging from one of the gravest crises in its post Independence history. The economic collapse of 2022 had led to shortages of fuel, food, medicines and electricity. Inflation soared, foreign reserves disappeared and long queues became part of daily life. The political upheaval that followed culminated in the resignation of former President Gotabaya Rajapaksa after mass public protests under the banner of the Aragalaya movement. The country was then governed by a leadership that spoke the language of reform and reconciliation but was widely perceived as lacking a direct popular mandate.

Sri Lanka’s past experience suggests that stabilisation and transformation cannot be treated as entirely separate stages. Postponing reconciliation until some future moment risks repeating the failures of the past. If transformation is endlessly delayed until a supposedly perfect moment arrives, there will always be new crises and new reasons for postponement. Minister Rathnayake’s contention that the government’s immediate priority has necessarily been stabilisation flows from the government’s awareness of the precarious situation the country is. Over the past two years, the government has succeeded to a significant extent in restoring economic and political stability. Inflation has reduced, shortages have ended and public institutions have regained a degree of functionality.

Guaranteed Changes

On the other hand, the country’s development continues to face challenges due to adverse global conditions, including disruptions caused by conflict in the Middle East and extreme weather events that have affected tourism, trade and the cost of living. The danger is that reconciliation may be indefinitely postponed in the name of stabilisation. This danger can be reduced if the government works proactively with the opposition and civil society to commence practical measures of transformation now rather than later. The participation of Opposition Leader Sajith Premadasa at the symposium, and the constructive nature of his comments, has strengthened the sense that bipartisan engagement on reconciliation may now be possible.

The urgency of transformation came through strongly in the presentations made by representatives of the Sri Lanka Tamil and Malaiyaha Tamil communities. ITAK parliamentarian S.Shritharan spoke of the frustration caused by unresolved post war issues in the north and east. He referred to disputes regarding land occupied during the war years, including controversies linked to Buddhist temples and state sponsored settlement activity in areas claimed by local communities. He also pointed to the continuing large scale presence of the security forces in the north and east nearly two decades after the end of the war. These grievances have remained central to Tamil political discourse since the end of the armed conflict in 2009. Families displaced by war continue to seek the return of ancestral lands. Civil society organisations in the north have repeatedly called for greater civilian control over local administration and a reduction in military involvement in civilian life.

Academic research and practical work on the ground have shown that reconciliation cannot be separated from questions of dignity, equality and justice. Former minister Mano Ganesan, leader of the Democratic People’s Front, focused on the longstanding problems faced by the Malaiyaha Tamil community. He spoke passionately about continuing housing shortages, landlessness and economic marginalisation, issues that have persisted since Independence. He also highlighted the devastating impact of recent extreme weather events on estate communities that remain socially and economically vulnerable. The condition of the Malaiyaha Tamil community remains one of the enduring social justice issues in Sri Lanka.

After Independence in 1948, a large proportion of them were denied citizenship and voting rights through legislation that rendered them stateless. Though citizenship rights were eventually restored, the social and economic consequences of exclusion continue to be felt generations later.

Many families still lack secure housing and land ownership despite their immense contribution to the country’s plantation economy. Minister Rathnayake’s responses to both these concerns were politically significant. He argued that recent political developments, including the declining influence of narrow ethnic politics across communities, indicated a major shift in public attitudes. According to him, the political ground has changed in ways that make it increasingly difficult for politicians who rely primarily on ethnic division and communal insecurity to retain public support.

Inter-Connected

There is evidence to support the assessment about the changing political grounding which sees future prospects in the resolution of long standing problems. . The economic collapse of 2022 affected all communities alike and generated a new politics centred on governance, anti corruption, accountability and economic justice. The Aragalaya protests brought together Sinhalese, Tamils and Muslims in a common demand for political change. Although ethnic grievances have not disappeared, the crisis created space for a broader understanding that the country’s future depends on cooperation rather than division. Opposition Leader Premadasa’s comments at the symposium reflected this changing political climate. He emphasised that national reconciliation could not be separated from economic justice and the need to address disparities between regions and social classes.v He also mentioned the need for civil society organisations to take this message to the community. This wider understanding of reconciliation is important because ethnic inequality and economic inequality have often reinforced each other in Sri Lanka’s history.

Academic studies have identified the denial of citizenship rights after Independence as a historic injustice that set back the Malaiyaha community for decades. The challenge now is to ensure that transformation becomes part of the stabilisation and development process itself. Practical first steps are both possible and necessary. The release of civilian lands still under state control, greater devolution of administrative authority, reduction of military involvement in civilian affairs, language equality in public administration and accelerated housing and land ownership programmes in the plantation sector are all measures that can begin immediately without waiting for a final stage of transformation.

The government’s recent commitment that provincial council elections will finally be held this year is therefore significant. These elections have been repeatedly postponed by successive governments. Holding them would not solve the ethnic conflict by itself. But it would signal a willingness to restore democratic institutions and share power in a meaningful way.

Sri Lanka has repeatedly postponed difficult reforms in the hope that a more convenient political moment would eventually arrive. But opportunities are invariably created and fought for instead of being provided as a gift by a benevolent government.

The present moment, shaped by the economic crisis and public demand for accountable government, offers a rare opportunity to move simultaneously towards stability, development and reconciliation. Provincial council elections can be the first meaningful step. But they must not be the last.

by Jehan Perera

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Researchers to shape new environmental policy framework

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Some of the researchers at the meeting

In a significant move aimed at steering Sri Lanka’s environmental governance towards a more science-based and evidence-driven path, the Ministry of Environment has initiated a new collaborative mechanism to integrate leading researchers into national policy formulation and conservation planning.

The initiative was discussed at a high-level meeting chaired by Dr. Dammika Patabendi at the Ministry of Environment on Tuesday, where top environmental scientists, wildlife experts and researchers were invited to contribute towards what officials described as a “strategic transition” in the country’s environmental management framework.

The discussions focused on strengthening the scientific basis of environmental conservation programmes and national policy decisions while creating a more research-friendly environment for academics and field scientists engaged in biodiversity and ecological studies.

Particular attention was paid to long-standing concerns raised by researchers regarding procedural and operational difficulties encountered when conducting studies in collaboration with the Department of Wildlife Conservation and the Forest Department.

Minister Patabendi stressed the need for environmental policies to be guided by credible scientific data rather than ad hoc administrative decisions, ministry sources said.

Among the key proposals discussed was the establishment of a streamlined mechanism that would reduce bureaucratic obstacles faced by researchers in obtaining approvals, accessing field sites and sharing scientific findings with state institutions.

The Minister highlighted the importance of building stronger partnerships between policymakers and the scientific community at a time when Sri Lanka is grappling with escalating environmental challenges including deforestation, biodiversity loss, human-elephant conflict, climate-related disasters and ecosystem degradation.

Environmentalists attending the meeting had also highlighted the urgent necessity of incorporating empirical research into national decision-making processes to ensure long-term ecological sustainability and better resource management.

The meeting brought together several of Sri Lanka’s leading environmental researchers and academics including Rohan Pethiyagoda, Saminda Fernando, Sewwandi Jayakody, Samantha Gunasekara, Dinidu Devapura, Himesh Jayasinghe, Manoj Prasanna, Mendis Wickramasinghe and Suranjan Karunarathna.

Director General of Wildlife Conservation Ranjan Marasinghe also participated in the deliberations.

Officials said the proposed framework is expected to pave the way for a more transparent, data-oriented and scientifically credible environmental governance structure capable of addressing emerging conservation challenges more effectively.

The government expects the new mechanism to support the implementation of practical and scientifically robust programmes aimed at safeguarding Sri Lanka’s ecological future while enhancing cooperation between state agencies and the country’s growing community of environmental researchers.

 

By Ifham Nizam

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Back home … for a special occasion

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Seven Notes: Sri Lankans based in Dubai – with Niluk (second from left)

Niluk Uswaththa, of Seven Notes fame, based in Dubai, surprised many when he and his wife Apeksha, turned up in Colombo, last week … unannounced.

Yes, they had a purpose in their surprise visit … to wish Apeksha’s mum for her birthday, which was on Monday, 18th May, and what a surprise it turned out to be!

In an exclusive chit-chat with The Island, Niluk said that the scene in Dubai is improving and Seven Notes do have work coming their way.

Since the members of Seven Notes are all employed (doing day jobs), they operate only on Saturdays and Sundays.

Niluk: Didn’t come prepared to perform, but obliged
friends in Galle

In fact, to get to Colombo for the birthday surprise (on Monday, 18th May), the band had to skip their 17th May, Sunday gig.

“Although it’s a short vacation, my wife and I are enjoying the setup here,” said Niluk, adding that they spent two days in Galle and that their next destination is Anuradhapura.”

Niluk didn’t come prepared to perform, but he obliged the crowd present, at a friend’s birthday celebrations, in Galle, singing and playing guitar.

They are scheduled to leave for their home, in Dubai, in the first week of June.

Seven Notes is an outfit made up of Sri Lankans and the band has been around for almost nine years.

Niluk came into their scene nearly seven years ago.

“When I went to Dubai, I had offers coming my way but it was Seven Notes that impressed me because of their acoustic style.”

The Dubai’s entertainment scene is showing clear signs of bouncing back and even levelling up in the next few months.

Niluk and Apeksha: Enjoying their short vacation

After a slowdown earlier this year due to regional tensions, shows and festivals are back on the calendar, and organisers say late 2026 could be the busiest concert season in years.

Time Out Dubai says “the 2026 concert calendar is filling up nicely” and “the city is ready to party once again” after some reschedules.

Dubai Summer Surprises in July brings retail activations, comedy nights, and indoor art exhibitions.

Organisers point to a backlog of postponed events that are being rescheduled for late 2026 and early 2027.

Yes, Dubai is calm on the surface but on alert. Life is mostly normal in the city, but there’s a “balancing act” as people watch for escalation.

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