Part 4 of 4: Maureen's story: copper, PLS and a question worth asking
Part 4 of 4: the final part of our metals and motor neurone disease series
Imagine your house has plenty of electricity coming in from the street, yet some of the lights upstairs keep flickering. The problem is not the supply. It is the wiring: how the power is carried from room to room, and whether it reaches the places that need it. Over the first three parts of this series, that is roughly the picture copper has painted in research on amyotrophic lateral sclerosis (ALS), the most common form of motor neurone disease (MND), the umbrella term for a group of conditions affecting the nerves that control movement. The question has rarely been how much copper there is. It has been where it goes, and whether it arrives.
In Part 1, we met SOD1, a protein that needs both copper and zinc to work: copper carries out the chemical reaction, while zinc helps keep the protein stable. The lesson there was that what counts is not simply how much copper or zinc we have, but what the body can do with them. Some ALS-associated forms of SOD1 bind copper less effectively than normal SOD1. Researchers have also suggested that copper could be a problem in ALS from both directions, too much and too little, sometimes even at the same time in different parts of the cell (Min, Sarlus and Harris, 2024). In Part 2, zinc and iron joined the story, and we saw how iron in the wrong place can quietly damage cells. In Part 3, we met caeruloplasmin. One of its main jobs is helping the body handle iron: it uses copper to change iron into a form that can bind to transferrin, the protein that carries iron through the blood. You can picture caeruloplasmin as a delivery van whose copper is both its cargo and its engine: it can only do its iron work once that copper is on board. Loading the copper is the job of ATP7B, the loading dock manager in the liver. We also met Morley Robbins and his question about whether problems with copper handling, rather than simply too little copper, deserve more attention. His wider explanation remains an unproven idea.
Part 4 takes those threads out of the laboratory and into the consulting room. This is one person's story. It is not proof of anything, and it is not a guide to treatment.
Meet Maureen
Maureen is in her seventies and lives with primary lateral sclerosis (PLS), a rarer, slowly progressing form of MND (Turner et al., 2020). PLS affects the upper motor neurones, the long nerve cells that carry instructions to move from the brain down into the brainstem and spinal cord. Over several years, Maureen noticed stiffness and problems with her balance and walking, and later her voice began to change, at times leaving her unable to speak. A second opinion confirmed the diagnosis. Maureen continues to be cared for by her neurology team.
Maureen did not feel the story ended with a label. When she looked back over her life, one theme kept surfacing, and it was copper.
Many years earlier, she had been severely anaemic and needed iron injections over several years. Later, a hair mineral test flagged high levels of copper. A recent study found that hair and blood mineral levels barely agreed with each other (Shahverdian et al., 2026), but the result set Maureen thinking.
With Dr Kirstie Lawton, PhD at the clinic, she then had a genetic panel that flagged her ATP7B gene, our loading dock manager from Part 3. Her blood tests showed copper and ceruloplasmin within the normal range. We also considered whether a histamine reaction to certain foods might explain her symptoms, but it did not fit her picture. Around the same time, Maureen cut back on copper-rich foods and felt that her voice became clearer. We should note that this is her own observation: her speech was not formally measured, and we cannot know what caused the change.
Maureen does not believe she has too much copper, or too little. She believes her body has a long-standing inefficiency in the way it moves and clears copper. She describes it as a "slow burn": not a sudden crisis, but something more like a candle left burning in a quiet room for twenty years. Nothing dramatic happens on any single day, but over time, she wonders, a small, steady inefficiency could add up.
It is an interesting idea, and it echoes the question Morley Robbins raises about copper dysregulation. At You Nutrition Clinic, we have also wondered whether it might help to explain why eating less copper seemed to help her. Picture the delivery vans waiting at the loading dock. If they are loaded slowly, some of the cargo may be left on the dock instead of being packed away. Copper in the blood that is not bound to ceruloplasmin is sometimes called "free" copper. Much of it is still held loosely by other proteins rather than being truly free, but researchers have suggested that this looser share could cause trouble even when the total amount of copper looks normal (Min, Sarlus and Harris, 2024). If someone was carrying a little more loosely bound copper than they could handle, eating less copper could, in theory, ease the load. This is an idea, not something Maureen's results show.
A word of caution
A low-copper diet is not an established treatment for PLS or any other form of MND, and it is not something we would usually recommend or have trialled with our clients. In fact, we more often find that people need support to get enough copper, which is part of why Maureen's experience stood out to us. Copper deficiency can itself damage the spinal cord, and recovery after treatment is often incomplete (Chen et al., 2024). Cutting out copper, taking copper supplements, taking high doses of zinc (which lowers copper) or using chelation treatments that pull metals out of the body should only ever be done under therapeutic supervision. Please talk to your medical team or a qualified practitioner before making these changes.
What her genes can and cannot tell us
Our genes are a recipe book, written in two copies, one from each parent. Across the population, the same recipe is often written with tiny spelling differences, called variants. Most make no difference at all to the finished dish. A few change it a little. Very occasionally, one ruins it.
Maureen carries one copy each of two common variants in her ATP7B gene. They are not the spelling mistakes that cause Wilson disease, the inherited copper condition we met in Part 3, and both are classified as benign in clinical genetic databases (ClinVar, n.d.a; ClinVar, n.d.b). They are also very common. In a large international database of genetic data, around half of people carry one copy of each variant, as Maureen does, and around a third carry two (Karczewski et al., 2020; GeneBe, n.d.a; GeneBe, n.d.b). In laboratory studies, these variants can slightly change how the ATP7B protein behaves, and one has been linked to a slightly higher share of loosely bound copper in the blood (McCann et al., 2019). But that research looked at groups of people and at the protein in the laboratory. It does not tell us how Maureen's own body handles copper, whether her variants affect her health, or whether she should change her diet.
As Part 3 explained, a panel like this is the start of a conversation, not a measure of how well someone handles copper. Blood tests have limits too. Measuring copper in the blood is like checking the water pressure at the mains: it tells you supply is coming into the building, but not whether the taps in each room are running. In mice with a change in a different copper gene, researchers found that copper fell in the brain and spinal cord while staying normal elsewhere in the body, and the mice lost some motor neurones in the spinal cord, although they did not develop obvious movement problems (Liddell et al., 2024). So normal blood results do not prove that copper is normal in every tissue. Equally, they are not evidence that something is wrong.
How could copper affect walking and speaking?
This is the heart of Maureen's question. If her body does handle copper inefficiently, could that explain the stiffness, the balance problems and the changes in her voice?
Walking and speaking both depend on the upper motor neurones. They carry instructions from the brain to the lower motor neurones in the brainstem and spinal cord, which pass them on to the muscles of the legs, throat and mouth. In Maureen's case, both walking and speech have been affected. Research points to three ways in which copper could, in principle, affect how well these long nerve cells work.
The first is energy. Copper is an essential part of the final step in the process that makes energy inside our cells (Gale and Aizenman, 2024). Nerve cells this long need a great deal of energy to keep their messages moving, and brain imaging shows that the areas most affected in PLS tend to be the ones with the highest energy demands (Kleinerova et al., 2026).
The second is protection. As Part 1 explained, copper is the working metal inside SOD1, one of the cell's main clean-up enzymes, which mops up harmful by-products of everyday energy production (Gale and Aizenman, 2024).
The third is insulation. Nerve fibres are wrapped in a fatty coating called myelin, much like the plastic around an electrical wire, which keeps signals fast and clean. In mice, a chemical called cuprizone, which binds copper, strips this coating away, although scientists still debate exactly how it does this, and copper loss may not be the main reason (Zirngibl et al., 2022).
We also know that a genuine shortage of copper can cause a stiff walk, brisk reflexes and unsteadiness on the feet (Chen et al., 2024). That does not mean copper is involved in PLS, but it does show that copper and the body's movement pathways are closely connected.
We have not found any studies testing these copper links in people with PLS, and Maureen's blood copper and ceruloplasmin is within the normal range (albeit it at the lower end of the range), so none of this shows for certain that copper is behind her symptoms. But it explains why her question is a reasonable one to ask.
The iron question
Maureen's history also raises a broader question, and it leads back to Part 3. Her iron injections were considered medically as the correct treatment for severe anaemia, and iron deficiency is itself harmful. If you have been prescribed iron, please don't discontinue this - instead, discuss any concerns with your doctor.
The question is a general one for research. Ceruloplasmin, loaded with copper, helps iron leave our tissues safely (Wang and Wang, 2019). In a rare inherited condition called acaeruloplasminaemia, people are born without working ceruloplasmin, and iron gradually builds up in the brain and other organs (Piperno and Alessio, 2018). As Part 2 described, iron left in the wrong place can damage cells (Ayton et al., 2025). Could more subtle differences in how copper is handled affect how well the body deals with large amounts of iron over many years? Morley Robbins has raised a similar idea, although it has not been tested in clinical trials. Maureen's ceruloplasmin results were (at the lower end of) normal, and nothing in her results suggests this happened to her. It is simply a question her history led us to ask.
What the research says
We have not found any studies looking directly at copper in PLS. However, there is research in ALS, and the two conditions are close relatives. Some researchers see them as different points along the same road rather than entirely separate destinations, and that debate is still going on (Turner et al., 2020).
The most direct study looked at spinal cord tissue donated after death by people with ALS (Hilton et al., 2024). Copper was lower in the area where motor neurones live. There was more ceruloplasmin than usual, yet its iron-handling activity was lower, which fits with not enough copper being available to it. SOD1, on the other hand, was working normally. In our terms, there were more vans on the road, but fewer of them carrying their copper cargo. A recent review of ceruloplasmin across brain conditions reached a similar conclusion: whether ceruloplasmin is working tells us more than how much of it there is (Pourhadi, LeWitt and Todi, 2026). Studies of tissue after death cannot tell us what caused the disease, and they do not tell us whether the same is true in PLS.
We also know that a genuine shortage of copper can look remarkably like ALS. In one published case, a man whose copper stayed low despite treatment developed an illness that came to resemble ALS (Benkirane et al., 2023). This does not mean that low copper causes ALS or PLS, but it is a reminder of how closely the two are linked.
Finally, there is a gap in the genetic research on PLS itself. Most people with PLS have no known genetic cause. Recent studies went looking for rare, single faulty genes, and found possible variations in only a minority of people (Manini et al., 2025; Schito et al., 2025). We have not found studies asking whether common, small variations in copper-handling genes, like Maureen's, might together make a difference.
None of this shows that copper is involved in Maureen's PLS, or in PLS generally. But it does show why the question is worth asking.
Recovery stories
Not every form of ALS follows the expected course, and the people whose illness surprises us can teach us a great deal. Diana Noland, a functional integrative nutrition practitioner in the United States, has described the case of a man with ALS who had needed a wheelchair, a feeding tube and breathing support. After adding a range of integrative approaches to his usual care, including copper supplementation, he regained the ability to walk, eat and speak over several years, although he still uses breathing support some of the time (Noland, Bergeron and Syrow, 2025). This was presented as a conference abstract, which the journal publishes without its own peer review, and because he used many approaches at once, it cannot tell us which, if any, made the difference.
Researchers at Duke University have compared people whose ALS reversed with people whose ALS progressed as expected. Those who improved were more likely to have taken several supplements, including copper (Harrison et al., 2018). This was not a treatment trial. It is an association, not proof that copper helped, and it tells us nothing directly about PLS.
Watch this space
There is still so much to discover. Maureen is continuing to look for answers with her neurology team, and to stay curious alongside that care.
What her story does is take the questions this series has been building and place them in one person's life. Copper has been widely studied in ALS. So, could the same kind of copper dysregulation also play a part in PLS, or in other neurological conditions? That is the open question Maureen's story leaves us with, and for now it is one that science has yet to explore. Sometimes a single story is most useful for the question it raises.
In Maureen's own words: "Trust your gut, look at your whole history, and don't be afraid to keep digging when something feels like a 'slow burn' that standard tests might overlook."
Supporting MND and ALS at You Nutrition Clinic
Dr Kirstie Lawton, PhD, MSc, FBANT, founder and Clinical Director of You Nutrition Clinic, leads a dedicated multidisciplinary team supporting people living with Motor Neurone Disease, Amyotrophic Lateral Sclerosis and Primary Lateral Sclerosis. Kirstie heads up the MND/ALS/PLS team alongside Kerry (RNTP), Deborah (RD) and Rachael (RN), with further support from Rae, our breath and movement practitioner. Together the team brings clinical nutrition, dietetics, nursing and movement support under one roof, with specialist experience in the particular nutritional and functional challenges that MND/ALS presents.
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Disclaimer: This article is for informational and educational purposes only and does not constitute medical advice. Always consult with a qualified, registered medical doctor (MD) for diagnosis and treatment decisions.
References
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