When a Gene Makes You Smell Like a Fish: And Other Amazing Tales about the Genes in Your Body

When a Gene Makes You Smell Like a Fish: ...and Other Amazing Tales about the Genes in Your Body by Lisa Seachrist Chiu. Oxford University Press; 2006; $27; 240 pages.

FROM THE DUST JACKET:

From the gene that causes people to age prematurely to the "bitter gene" that may spawn broccoli haters, this book explores a few of the more exotic locales on the human genome, highlighting some of the tragic and bizarre ways our bodies go wrong when genes fall prey to mutation and the curious ways in which genes have evolved for our survival. Lisa Seachrist Chiu offers here a smorgasbord of stories about rare and not-so-rare genetic quirks -- the gene that makes some people smell like a fish, the Black Urine Gene, the Werewolf Gene, the Calico Cat Gene. We read about the Dracula Gene, a mutation in zebra fish that causes blood cells to explode on contact with light, and suites of genes that also influence behavior and physical characteristics. The Tangier Island Gene, first discovered after physicians discovered a boy with orange tonsils (scientists now realize that the child's odd condition comes from an inability to process cholesterol). And Wilson's disease, a gene defect that fails to clear copper from the body, which can trigger schizophrenia and other neurological symptoms and can be fatal if left untreated. On the plus side, we read about the Myostatin gene, a mutation which allows muscles to become much larger than usual and enhances strength -- indeed, the mutations have produced beefier cows and at least one stronger human. And there is also the much-envied Cheeseburger Gene, which allows a lucky few to eat virtually anything they want and remain razor thin.

While fascinating us with stories of genetic peculiarities, Chiu also manages to explain cutting-edge research in modern genetics, resulting in a book that is both informative and entertaining. It is a must read for everyone who loves popular science or is curious about the human body.

WHAT THE CRITICS SAY:

Publishers Weekly: Although Chiu uses a catchy title, cute jokes, and soft watercolor illustrations by her mother to disguise this book as popular science, she has produced a rigorous and detailed survey of the most recent developments in human genetics; a "Genetics Primer" is appended, and many readers will no doubt need it. The first chapter, on a woman who smelled so badly of fish she had to take a three-month leave of absence from work, seems at first the usual, chatty fare of much popular science writing. Within a few paragraphs, however, Chiu has launched into a complex discussion of gene mutation and enzymes. Chiu writes best in her detailed accounts of these genetic oddities, but the names Chiu and others have given the genes responsible ("The Cheeseburger Gene," "The Werewolf Gene," "The Calico Cat Gene") often belie their seriousness, a problem echoed in Chiu's personal anecdotes, which seem to serve less as relevant commentary than as deliberate bids for a larger readership. Chiu's greater contribution is in her willingness to trust her audience with explanations of genetics research that are long, dense, complicated, and surprisingly accessible.

ABOUT THE AUTHOR:

Lisa Seachrist Chiu is a journalist and writer who has covered the cutting edge of genetics, medicine, and molecular biology for more than a decade. She's been published in United Press International Syndicate papers, Science, Science News, BioWorld Today, Discovery.com, and the Howard Hughes Medical Institute. She lives in Washington, D.C.

A CONVERSATION WITH THE AUTHOR:

When I phoned Lisa Seachrist Chiu at her home in Washington D.C., she and her husband had just returned from a trip to the park with their 20-month-old daughter, Anya. As Lisa and I spoke on the phone, father and daughter could be heard in the background, making preparations for a midday nap. "Were you expecting at the time you were writing your book?" I asked. "As a matter of fact, I was. I would say that writing a book on genetics while you are pregnant is probably not the best thing to do. It's a little too much information."

"Where did the notion for this book originate?"

"It started as an idea by an agent. The agent sold the idea to a friend of mine who got the book contract and ultimately decided that he didn't want to do it. I had been working for a dot-com, and the dot-com went 'poof,' so I was in a place where this was a reasonable thing for me to consider doing. Then, through a whole host of reasons, the book took longer than I expected. There were changes in editors. At one point I had to re-do the proposal. There was a lot of silliness. Then, because it was not my idea, originally, there were additional challenges.

"Originally the book was sold as a bunch of interesting little stories about genes. The idea was that there would be 40 to 60 of them. But Oxford felt there needed to be some unifying theme, so I organized the stories around major concepts like dominant and recessive genes. You could nearly teach a genetics course with the stories that I have."

"What background did you bring to the topic?"

"I have a master's degree in Biochemistry from Duke, so I have a lot of science background. I'm also the former Washington editor for a biotechnology newspaper called BioWorld Today, so this kind of stuff was really old hat for me."

"When you thought about writing this, did you identify an ideal reader and determine what sort of background they would bring to the piece, so that you knew how much information to provide?"

"You know, I probably should have. I first identified the very basic things that you need to know in order to understand these stories. I tried to put all of that into the Genetics Primer in the appendix. Originally, that section was at the very beginning of the book. It became clear right away that that just wouldn't work. Nobody wants to be schooled right off."

"It sure didn't take me long to scoot to the Primer as I read Chapter 1."

"That's just the thing; you leave it as an option and then repeatedly remind people it exists.

"I also repeated information, intentionally, from chapter to chapter. When I pick up a book that has as many little discrete chapters and stories as this one does, I'm not going to read it straight through. I'll read a section then look at the next section and decide. So, I needed to repeat some of the technical aspects so that readers could understand the various sections independently. I didn't expect anybody to remember from one chapter to the next.

"If I were going to spend another few months on it, I would spend more time working with my mother to include more illustrations. There are so many more we could have done had my brain been wrapped around the idea. Originally, I thought about using images from scientists, but they were too off-putting. My mom does watercolors, so I thought it was a good idea."

"Does your mom have a background in the sciences?"

"No, she's a theater major."

"How did you explain things to her in such a way that she was able to illustrate them?"

"First, I would give her the long description, taking her step by step. She would ask, 'What is the most important part of all this? What are we trying to show?' As we talked, the picture came clearer. Fortunately, I communicate well with my mother."

"I have to ask, did you ever have to send her back to the drawing board?

"Yes. It was fine, because she is remarkably fast. At one point I flew her down for two weeks. Of course, at that point, there was a granddaughter involved, so it was pretty easy to get her to come down."

"In what ways were you encouraged, as a girl and a young woman, to pursue an interest in the sciences?"

"I was always expected to do well in school. There was never any doubt. I was just expected to, and it didn't really matter what the subject was. Because there was that expectation, and I was not a particularly confrontational or contrary child, I wanted to do well.

"Every family has their stories. My grandfather's father died very young. He was a veterinarian and was killed by a horse. Because of that, and a number of other circumstances, my grandfather was unable to go to college, even though he had received a partial scholarship. Because his father couldn't go to school, it became a terrifically important thing, when my father was growing up, that he and his sisters go to school. Going to college was very important in my family as well, because my grandfather didn't get to go."

Suddenly, there is a click on the line, and Lisa's daughter, fresh from her shower, chimes in with a series of distinct syllables, "Ray, me, me, me." As Dad whisks Anya off, I reflect on how much I enjoyed my own daughters at that age. "Once they learn to speak, they have opinions, and it's not quite as fun." I muse.

"She's beginning to have opinions already. She's a very early talker. She's 20 months old and she speaks in sentences. You can hear her coming down the stairs."

"How important are genetics in something like early verbal ability?"

"Language has been one of those difficult things to pin down. So far they seem to have identified just one gene related to it. If you have a mutation in that gene, you have problems with actual speaking and with syntax and all sorts of verbal abilities. Other than that, they really don't know what is involved in language acquisition and skills. Knowing about the one gene gives you a place to look for others that have a smaller effect.

"My mother says that I was also an early talker. So, perhaps there is some genetic component to it."

"How did you go about selecting the human stories you use to illustrate the effect of genetic disorders?"

"The story about fish odor syndrome, from which my book takes its title, made a splash in local newspapers and magazines when it was first identified. There have, in fact, been two symposia on the disorder. I went to one, and so was already familiar with the story prior to starting the book.

"Since the 1960s there has been speculation that Abraham Lincoln had Marfan Syndrome. I knew that women's volleyball legend Flo Hyman had also suffered from Marfan Syndrome. I thought that because she was such an important volleyball player, and was so important for women's sports in the 1980s, that this would be a good one to start with.

"Marfan's is an interesting gene because it has so many potential effects. The specific genetic disorder interferes with a very important and widely used protein, so it can manifest itself in any number of ways. You can have problems with the arches of your feet, the main blood vessels of your heart, or the lenses of your eyes. It can also cause you to have lengthy bones and to be very flexible. Marfan Syndrome is a great way to explain the notion of pleiotrophy -- the concept that one gene ends up having myriad effects because it involves such a basic process.

"I've always been personally interested in Marfan because I'm an extremely short human being, but my father's father (the veterinarian) was 6'8". He married a woman who was 4'8", and we've been short ever since. One of the interesting things about him, though, was that he sat short. When he sat down he looked to be the same size as everyone else, but when he stood up he was all legs and arms. Looking at him, I have to wonder if he had Marfan Syndrome. And, if so, had he not been killed by that horse, would he have died an early death anyway? Maybe he would have had a weak aorta."

"You say that the first condition recognized to be genetic in nature, Black Urine Disease, was identified as early as the 1580s. You also note that the discovering physician, Scribonius, was a leading proponent of the water test for witches. It's heartening that this child, whose urine turned inky black when it was exposed to the air, was never tried as a witch himself. Or was he?"

"There wasn't any mention of witchcraft. I guess because the child had no other symptoms -- he wasn't having fits, and nobody was having problems around him -- they assumed he wasn't a witch.

"That does bring up an important point, though. When the Human Genome Project was being started, I went to a press conference with Francis Collins. One of the concerns expressed early on was that people would be stigmatized once something was identified in their genetic makeup. Collins said that it was possible that people would, indeed, be stigmatized, initially. He also said how wrong that was to do, because the best estimates were that all of us carry between seven and nine really bad genes. Whether or not they get expressed depends on which genes they are -- which is luck of the draw -- and then what kind of environment you find yourself in."

"I can't help but think that genetics is not the field for persons who seek ultimate answers. It seems that each answer that's been found has just led to a thousand more questions."

"It's not a field for somebody who's looking for black and white. You get incremental answers. People seem to think that once you've identified a gene, you can move right to creating a drug or gene therapy. But, it's not that simple, and it's not that straightforward. Biological systems are enormously complex. Changing one thing in a cell can affect so many other things.

"The one thing that frustrates the general public more than anything else is the lack of certainty. You hear people say, 'Just tell me what to eat so I live well.' But it isn't that simple.

"A promising outcome of recent genetic research, though, has to do with SNPs [single nucleotide polymorphisms]. These are tiny variations that cause individuals to respond to various drugs in unique ways. By identifying a person's SNP profile, you can select drugs that are effective and avoid those that aren't. I think this personalized medicine is going to provide some of the answers people have been seeking from genetics research."

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