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About the Institute

Profile of Brian Robinson

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Dr. Brian Robinson

By: Alena Boczek

Dr. Brian Robinson, PhD

  • Senior Scientist, Genetics & Genome Biology
  • Professor, Biochemistry and Paediatrics, University of Toronto
  • Canada Research Chair, Vascular and Metabolic Biology

1. Where are you from?/Where did you study?
I was born in Derby, in the United Kingdom and I studied at the University of Bristol in England for my Bachelor of Science and for my PhD. I first came to Canada in 1968 to do a fellowship at the University of Toronto.

2. What are you researching right now?
We are a lab that tries to figure out the origins of genetic diseases in children that are related to energy and metabolism. We specialize in a group of diseases which are called mitochondrial diseases. In these diseases, the mitochondria, which are the power plants of the cells become defective, usually because of genetic reasons. The consequence of that can be anything from mild exercise intolerance, to severe debilitating acidosis (too much acid in the body) in the neonatal period followed by neurodegeneration. So the spectrum of disease is extremely wide and it’s very difficult to diagnose. Over the years my laboratory has put together a series of diagnostic tests and we apply them to children who are suspected of having these mitochondrial diseases to determine if that indeed is the case. We perform this testing is not just for kids who come into SickKids, we do it for many of the other centres within Canada. We get samples from British Columbia, Manitoba, Nova Scotia, and we get samples from all around the world, including Europe, the Middle East, and South America. We’re sort of an international referral centre for some of these diseases. That’s the basis of how we liaise with the hospital.

As we detect various types of disease, we try to break that down into a molecular-genetic diagnosis. We do this so we can help families make informed decisions, about future pregnancies for example, depending on what kind of genetic disease the children are suffering from. Also, the diagnosis (discovering which gene is the culprit for the disorder) will sometimes lead you toward a specific form of treatment. This can vary from dietary treatment, cofactor treatment, and sometimes drug treatment – it all depends on what kind of defect they actually have.

In addition to that, we do some very basic work in the lab, trying to find out how mitochondria, these little power plants, replicate themselves. We’re interested in some special proteins that protect the Ribonucleic Acid (RNA) within the mitochondria as they’re replicating.

The other thing we’ve been doing is trying to correlate the difference between structure and function of the mitochondria from an ultrastructure point of view. We work with Cameron Ackerley, an electron microscopist to determine how these various defects in the mitochondria impact the structures you see when you look at electron micrographs of muscle or cells grown from children with the defects. Sometimes you can see quite specific architectural abnormalities in the mitochondria that help you make the diagnoses before a lot of money is spent doing genetic analysis.

3. Who is your all-time favourite scientist, and why?
There are two Nobel Prize winners who I interacted with in my younger days who impressed me greatly. They were Sir Hans Krebs, the discoverer of the Krebs cycle and a legend in metabolic circles, and Peter Mitchell who basically figured out how mitochondria generate energy. Krebs received his Nobel Prize in 1953 and Mitchell got his in 1978. I was greatly impressed by them because they were not overbearing. They were very thoughtful, retiring people who were obviously just interested in science, so I always thought that they were very special people. And they of course impacted on the field that I was working in.

4. What in your opinion is the single most important scientific breakthrough, and why?
In the years that I’ve been a scientist, the Dideoxy method of sequencing DNA has to be the most important. The guy that invented it was Fred Sanger. Sanger was very unusual in that he got two Nobel Prizes for science. He got one for discovering the structure of insulin, which he did in 1958, and then later on in his career he produced this brilliant method for sequencing DNA. Basically, if he hadn’t come up with this method, I don’t think the human genome would have been sequenced as fast as it was. That one technique that he developed led to so much else that was produced.

The breakthrough was developed as a technique to solve a problem – not as a hypothesis. This technique for performing DNA sequencing was an absolutely brilliant development at the time. As far as I’m concerned, it has only recently been surpassed. Thirty years later there are some more modern methods creeping in, but for thirty years it basically dominated the field in terms of how to do things.

5. What are your major interests outside the lab?
I am a collector of things. I have a lifelong interest in English, Scottish, and Irish Folk music. I play the guitar and the banjo, and I have a huge collection of folk material. That’s weekends.

I also have a pretty comprehensive collection of postcards, which has been a hobby of mine for the last twenty or thirty years. They’re mostly of places that I know well, usually in Ontario, dating back over the years. And, I even have a collection of cards from The Hospital for Sick Children! I have some that date back to the old building that was on College Street. Finally, I’ve always been interested in steam engines. I haven’t parlayed that into much of a hobby, except that I collect model railway engines. There’s one model railway engine here at SickKids – I always keep one in the office.

6. Why science?
How did I end up in the world of science? Well, my mother and father bought me a chemistry set when I was about nine years of age. Unlike the chemistry sets nowadays, which are just baking powder and vinegar, there was some real good stuff in there. With some ingenuity, you could make some pretty fierce reactions resembling volcanoes and rockets or whatever you wanted to do.

When I got my hands on my first chemistry book at school, when I was 11, I took it home and read it from cover to cover. I was so interested in how these chemical reactions take place. I guess that’s where my interest in science started and it parlayed itself into me taking science courses in school, which lead me to pursue a degree in biochemistry. A lot of it was accidental, but it just so happened that I had some very good teachers in university that inspired me to do some post-graduate work. I lucked-out and got into a good lab that studied energy metabolism and that’s how I got started in mitochondria. I’ve been basically working on that subject for the whole of my career.

7. Why SickKids?
That was a bit of serendipity. After I did my fellowship at U of T in the medical sciences building, I went back to England. There, I taught at the University of Sheffield for three years… and I hated it. After being in Canada, it felt like the sun never shone – it was always raining – and people were generally in a bad mood. When it was suggested that I apply for a position at SickKids that had become vacant, I jumped at it. In 1973, I came back to SickKids and I’ve been here ever since.

Again there’s a strange history because I was originally hired to investigate the phenomena of neonatal hypoglycemia, in other words babies that have low blood glucose, and this really did not turn out to be a problem with energy metabolism. Though we discovered the cause was unrelated, I suddenly realized there were patients in the hospital who did have defective mitochondria and abnormal energy metabolism. As a result, I teamed-up with one of the metabolic physicians and started to investigate disorders of energy metabolism and that’s what I’ve been doing ever since.

8. What is the most controversial question in your field right now?
That is a hard one. One of the burning questions is: Does the process of aging have anything to do with the deterioration of energy metabolism? To clarify, does the deterioration of energy metabolism come first and then you age? Or do you age and then, as a result, your energy metabolism goes down? There’s a lot of debate about this.

Although our lab hasn’t been directly involved in a lot of these experiments, I did write a critical review on this a few years ago which is probably one of the most highly cited reviews in the literature (it’s been cited more than 500 times!). In the review, we put together a hypothesis of how the aging process might interact with mitochondria to produce defective energy metabolism. I think that’s going to be an interesting subject that people are going to be debating for some time.

9. What are you reading right now?  
I read a lot. I read two types of books, basically. I read detective novels; I really like a good whodunit. The last one I read was by my namesake, Peter Robinson. He’s an English writer living in Canada, and he wrote a book called Before the Poison, his latest book, which was really good. I probably read about 15 or 20 whodunits a year. I guess that feeds into my scientific, inquisitive nature. I like to guess who did it before I get to the end of the novel.

I also like some non-fiction books. Just after Christmas, I read a book called At Home by Bill Bryson, in which he describes the rooms in his house. He discusses how all the different rooms we use every day (the hall, the bathroom, the nursery) evolve into what they are today compared to what they were 100, 200, 300 years ago.

Another good one I read after Christmas was a book by Robert Buckman – he passed away just before Christmas. He was on a flight back to Toronto from England and he died in his sleep. He was a medical doctor and one of his beliefs was that humour is a really great thing you can use to deal with serious illness. I think he had a lot of good things to say about that. This particular book was called Not Dead Yet. He had written this about his early life as a medical student and how he got into research. It was very funny; he really makes you laugh.

10. If you could give one piece of advice to someone considering a research career, what would it be?
I would say, try and hitch your wagon to a star. In other words, get into the best laboratory you can possibly get into. The people in those labs have an immense amount of experience and you can really learn a lot from them. These people have so much to teach, and, often, the people coming out of the top labs get great jobs and many research grants because they’ve learned under the best.

March 2012