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- <text id=92TT0361>
- <title>
- Feb. 17, 1992: The Saga of The Vicious Gene
- </title>
- <history>
- TIME--The Weekly Newsmagazine--1992
- Feb. 17, 1992 Vanishing Ozone
- </history>
- <article>
- <source>Time Magazine</source>
- <hdr>
- SCIENCE, Page 72
- The Generational Saga of The Vicious Gene
- </hdr><body>
- <p>Researchers find a surprising type of heredity that can make
- a defect more serious in a child than in the parent
- </p>
- <p> For more than a century, scientists have built upon the basic
- principles of heredity that Austrian monk Gregor Mendel gleaned
- from his painstaking studies of garden peas. One of the most
- strongly held beliefs has been that genes--whether normal or
- abnormal--are passed from generation to generation
- essentially unchanged. Now that assumption is being challenged.
- Last week scientists announced that in people with a form of
- muscular dystrophy, they had identified a segment of DNA that
- can lengthen substantially with each succeeding generation. Most
- disturbing, as the fragment lengthens, the illness becomes more
- severe. "This is not your garden-variety genetic defect," says
- Dr. Leon Charash, who chairs the medical advisory committee of
- the Muscular Dystrophy Association.
- </p>
- <p> The startling discovery, reported in Nature by an
- international trio of research teams, marks only the third time
- such a genetic phenomenon has been found. Last year researchers
- revealed a similar process in two much rarer inherited diseases:
- fragile X syndrome, a form of mental retardation; and spinal and
- bulbar muscular atrophy, a wasting disease.
- </p>
- <p> While forcing scientists to revise their thinking about
- heredity, the findings are also raising ethical quandaries. "It
- now appears we can identify people who may be asymptomatic but
- whose risk for transmitting a devastating illness is very high
- compared with the rest of the population," observes geneticist
- David Housman of the Massachusetts Institute of Technology, a
- member of one of the research teams. "Should they be informed?"
- A man or woman with such a defect will have to consider the
- brutal fact that not only is there a fifty-fifty chance that a
- child will inherit the illness, but also that the disease may
- be progressively worse in that child, the grandchildren and the
- great-grandchildren.
- </p>
- <p> Over the years, researchers have discovered that the DNA
- that makes up the 46 chromosomes in the human cell is not as
- stable as once thought. Mutations in DNA have long been known
- to occur, but they usually involve relatively small changes in
- genetic material. For example, between parent and child, there
- may be a switch in the sequence of nucleotide bases that are the
- building blocks of DNA. Sometimes an entire gene can jump to
- another place on a chromosome. "But you don't usually see a big
- increase in the absolute number of bases within a single gene,"
- says Greg Lennon, a geneticist at Lawrence Livermore National
- Laboratory in Livermore, Calif., and a member of one of the
- teams that made last week's announcement. Moreover, mutations
- tend to occur at a slow pace. "The rate is so low from one
- generation to the next--maybe 1 in 10,000--as to be
- negligible," notes M.I.T.'s Housman.
- </p>
- <p> In myotonic dystrophy, the most common form of muscular
- dystrophy, the change can be far from negligible: a fragment of
- DNA on chromosome 19 appears to repeat itself more frequently
- with every generation. Just what triggers the repetition is a
- mystery. Researchers surmise that a hitch occurs while DNA is
- being copied in the cell, much as the same bar of music repeats
- on a scratched record. The DNA repeat gets worse with each
- generation, just as with each playing of a flawed record, the
- music stutters for a longer period. "Presumably the replication
- error occurs in the sperm or egg before conception," says
- molecular geneticist Pieter de Jong, who headed the Livermore
- team.
- </p>
- <p> Scientists expect to find more stuttering genes. "Any time
- a disease gets worse through generations, we're going to
- suspect that this happens," notes Lennon. Researchers are also
- intrigued by the possibility that gene growth occurs as cells
- replicate in the body during a person's lifetime. That would
- have implications for ailments such as cancer. The search begun
- by Mendel for the secrets of heredity is far from complete.
- </p>
- <p>-- By Anastasia Toufexis
- </p>
-
- </body></article>
- </text>
-
-