Scientists say they have made a breakthrough in understanding the cause of both motor neurone disease and a rare form of dementia.
They have discovered that a protein called FUS causes brain cells to die in both conditions.
The researchers, from Cambridge and Toronto, said they were cautiously optimistic their findings could one day to lead to improved treatments.
The study is published in the journal Cell.
Motor neurone disease (MND), also known as ALS, is a progressive and terminal disease that damages the function of nerves and muscles, resulting in severe damage to the brain and spinal cord.
It affects up to 5,000 adults in the UK at any one time.
Frontotemporal dementia is a form of dementia that causes changes in personality and behaviour, and language difficulties.
Both conditions are caused by the death of brain cells and this study shows that a similar mechanism is involved in each.
'Jelly with fruit'
The researchers looked at a protein called FUS which is vital for nerve cells to work properly.
It is able to change state between oily droplets and more solid jellies and in both diseases it can become trapped in its jellied form.
Synapses – the point where two nerve cells meet – are highly active and need to produce a lot of new proteins in order for messages to be passed from one cell to the next.
FUS grabs the instructions for those proteins as it becomes a jelly and releases them as it becomes an oil.
Prof Peter St George-Hyslop, from the University of Cambridge, told the BBC: "In the jelly phase it is like a fruit salad – jelly with bits of fruit – that move around the cell and the bits of fruit are the machinery needed to make proteins."
But if FUS stays jellied then that machinery is not released and the brain cell fails to function and eventually dies.
In motor neurone disease, the FUS protein can be mutated and more prone to becoming stuck in a jellied form.
In frontotemporal dementia, the problem is with other enzymes which help FUS change state, and they tip the balance towards the jelly.
Prof St George-Hyslop says these enzymes could be the key to treating both conditions.
"What we didn't know is really how this process is governed and that's the crucial piece of information," he said.
"So can we use these enzymes that control the process to inhibit or accelerate it – that's the big step forward."
Prof St George-Hyslop said he was "cautiously optimistic" this would be possible, however he was clear this would not help patients today.
The FUS protein is involved in a delicately balanced system and finding a treatment for MND and dementia will not be easy.
For example, a drug that made FUS too oily would cause as many problems as when it becomes too jellied.
The latest discovery was made using human cells that resembled neurons as well as neurons from frogs.
The scientists hope future research will discover drugs that can help FUS revert to its oily state.
"It now opens up a new avenue of work to use this knowledge to identify ways to prevent the abnormal gelling of FUS in motor neurone disease and dementia," added Prof St George-Hyslop.
The research was funded by the Wellcome Trust.
Dr Giovanna Lalli, from Wellcome's Neuroscience and Mental Health team, said: "This study provides important new insights into a fundamental process underlying neurodegeneration.
"Through their research, the team have uncovered promising new ways to tackle these diseases."
Burkina Faso: Growing Violence Threatens Health Care
Away from the worlds attention, Burkina Faso has been slipping into violence. In less than a year, t..
Away from the worlds attention, Burkina Faso has been slipping into violence. In less than a year, the number of displaced has increased fivefold, from 50,000 last December, to 270,000 in August. As ever, the most vulnerable suffer most: the very young, and the very old.
When Alidou Sawadogos elderly mother fell ill, he faced a long and dangerous journey to get treatment for her.
“When she collapsed, a friend called me,” he explains. “By the time I arrived she was already unconscious. I decided to take her to the health center and luckily someone who had a motorcycle helped me. Because of the violence many people who are sick wait at home and die. Everyone is afraid of taking the road to the health center in Barsalogho.”
Across Burkina Faso, the rising insecurity has forced over a hundred health centers to close, or to limit their work. Half a million people now have little or no access to health care. Dedicated health workers, among them Dr Bertrand Dibli in Barsalogho, are struggling to meet the needs, and to stay safe themselves.
“This is one of the few health centers that isnt closed,” he says. “We dont have enough equipment. And the insecurity has caused huge anxiety among health workers. Even coming here to Barsalogho is a huge challenge because the route is so dangerous.”
The ICRC has been working to support Burkina Fasos health professionals, with medical kits, and vaccination campaigns. During his visit to the country, ICRC President Peter Maurer expressed his concern at the multiple challenges facing Burkina Fasos people.
“We are very concerned,” he said. “Very worried about the upsurge in violence, its a vicious circle that is trapping the civilian population between armed groups.”
“We also see,” Mr Maurer added, “that it is not only the violence that is affecting the country, it is also under development, and climate change. Together with the violence that is obstructing the health services, its an accumulation of factors.”
And so the ICRC – jointly with the Burkinabé Red Cross – is also delivering food to the displaced, and helping to improve access to water supplies. All of this, says nurse Jeanette Kientega, is desperately needed by a population uprooted by conflict, and denied access to basic health care.
“By the time they are able to get here, it is often too late” she says. “Sometimes we can help, but if they have already been ill a long time, it is difficult. We try to do what we can.” (more…)
World Bank and WHO Statement on Partnership & Deployment of Financing to WHO for Ebola Response in DRC
WASHINGTON, August 23, 2019—The World Bank and the World Health Organization (WHO), along with the G..
WASHINGTON, August 23, 2019—The World Bank and the World Health Organization (WHO), along with the Government and other key partners, are working in close partnership on the Ebola Crisis Response in the Democratic Republic of the Congo (DRC). Central to this partnership is the assessment of the financing needs, and deployment of resources, with the goal to put an end to the current deadly outbreak.
The World Bank is today announcing that US$50 million in funding is to be released to WHO for its lifesaving operational work on the frontlines of the outbreak. The WHO is announcing that this US$50 million in funds will close the financing gap for its emergency health response in DRC through to the end of September 2019, and is calling on other partners to mirror this generous support in order to fund the response through to December.
The funding comprises US$30 million from the Pandemic Emergency Financing Facility (PEF) and US$20 million from the World Bank. The US$50 million in grant funding is part of the larger financial package of approximately US$300 million that the World Bank announced last month to support the fourth Strategic Response Plan for the DRC Ebola outbreak.
“WHO is very grateful for the World Banks support, which fills a critical gap in our immediate needs for Ebola response efforts in DRC, and will enable the heroic workers on the frontlines of this fight to continue their lifesaving work,” said Dr. Tedros Adhanom Ghebreyesus, Director-General, World Health Organization. “We keenly await further funding from other partners to sustain the response through to the end of the year.”
The DRC government, working in collaboration with the World Bank, WHO, and other key partners, has finalized the Fourth Strategic Response Plan (SRP4), which outlines the total resources needed for the DRC Ebola Crisis Response from July to December 2019. The financing announced today is part of the World Banks previously announced financial package of up to US$300 million and covers over half of SRP4s needs, with the remainder requiring additional funding from other donors and partners.
“The World Bank is working closely with WHO, the Government of DRC, and all partners to do everything we can to put an end to the latest Ebola outbreak,” said Annette Dixon, Vice President, Human Development at the World Bank. “The partnership between our organizations and the Government is critical for responding to the emergency as well as rebuilding systems for delivery of basic services and to restoring the trust of communities.”
The Government of DRC requested US$30 million from the PEF Cash Window to be paid directly to WHO. The PEF Steering Body approved the request bringing the PEFs total contribution to fighting Ebola in DRC to US$61.4 million. The PEF is a financing mechanism housed at the World Bank; its Steering Body is co-chaired by the World Bank and WHO, and comprises donor country members from Japan, Germany and Australia. The quick and flexible financing it provides saves lives, by enabling governments and international responders to concentrate on fighting Ebola—not fundraising.
Borno State launches first Malaria Operational Plan, reawakens fight against malaria
Maiduguri, 13 August 2019 – Following recommendations from malaria interventions in Borno State Nige..
Maiduguri, 13 August 2019 – Following recommendations from malaria interventions in Borno State Nigeria, the Malaria Annual Operational Plan (MAOP) was developed and launched on 08 August 2019 with technical support from the World Health Organization (WHO) and partners. Aligned to the National Malaria Strategic Plan (2014 -2020), MAOP was developed through a broad-based stakeholders workshop involving malaria stakeholders, reviewed on different thematic areas and endorsed by the Commissioner for Health and Permanent Secretary, Borno State Ministry of Health.
Speaking during the launch, the Borno state Malaria Programme Manager, Mr Mala Waziri described the MAOP as the first to be endorsed and disseminated in Borno State. “WHO has made us proud by supporting the first ever Malaria Operational Plan right from development, review, printing to dissemination.”
Dr Ibrahim Kida, the Ministerial Secretary Borno State Ministry of Health and Incident Manager of the state, described the launch as “an historic event as stakeholders across the health sector made commitments to use the document as an implementation guide for all malaria programs”. The plan was also described as an advocacy tool for planning domestic funds mobilization.
The MAOP has seven objectives among which are: provide at least 50% of targeted population with appropriate preventive measures by 2020; ensure that all persons with suspected malaria who seek care are tested with Rapid Diagnostic Test (RDT) or microscopy by 2020 and all persons with confirmed malaria seen in private or public health facilities receive prompt treatment with an effective anti-malarial drug by 2020.
The MAOP will further ensure that at least 50% of the population practice appropriate malaria prevention and management by 2020, ensuring timely availability of appropriate anti-malarial medicines and commodities required for prevention, diagnosis and treatment of malaria in Borno State by 2020.
In addition, it seeks to ensure that all health facilities report on key malaria indicators routinely by 2020 and finally strengthen governance and coordination of all stakeholders for effective program implementation towards an A rating by 2020 on a standardized scorecard. These strategic objectives have specific targets and the MAOP takes into account the humanitarian response.
“Malaria remains a leading cause of poor health in Nigeria. According to the 2018 WHO Malaria Report, 53million cases are recorded annually in Nigeria, roughly 1 in 4 persons is infected with malaria contributing 25% of the global burden,” says Dr Nglass Ini Abasi, WHO Malaria Consultant for the North East.
“Furthermore, 81,640 deaths are recorded annually (9 deaths every hour), which accounts for 19% of global malaria deaths (1 in 5 global malaria deaths) and 45% malaria deaths in West Africa. The Nigeria Malaria Strategic Plan (NMSP) 2014-2020 has a goal to reduce malaria burden to pre-elimination levels and bring malaria-related mortality to zero and WHO is working assiduously with Government to ensure the burden is reduced accordingly.”
Results from WHO’s Early Warning, Alert and Response System (EWARS) week 30 report from 223 sites, (including 32 IDP camps) show that malaria was the leading cause of morbidity and mortality accounting for 35% of cases and 46% of reported deaths. In addition, results from the Nigeria Humanitarian Response Strategy (NHRS 2019-2021) indicate 7.1million people are in dire need of healthcare and 6.2million are targeted for immediate attention.
Despite recent improvements, insecurity remains a challenge limiting access to the functional health facilities. Easily preventable and treatable diseases such as malaria, acute respiratory infection and diarrheal diseases account for the greatest proportion of morbidity and mortality among the vulnerable population. Furthermore, Malaria is endemic in North East Nigeria and the transmission is perennial with a marked seasonal peak from July to November every year. (more…)
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