June 1995 The gene that causes a condition known as pseudoachondroplasia, a form of dwarfism, has been identified by a research team led by Dr. Jacqueline Hecht of The University of Texas-Houston Health Science Center. Identification of the gene will allow scientists to make preliminary inroads into understanding and someday curing the condition. The discovery is reported in the July 1 issue of the journal Nature Genetics. The abnormal gene is located on chromosome 19 and is called the cartilage oligomeric matrix protein (COMP) gene for its role in making this particular form of protein, which acts as a building block in the body. The COMP gene is normally present in all individuals, and mutations in this gene cause pseudoachondroplasia. "With the identification of the pseudoachondroplasia gene we plan to study the normal interactions of this protein in order to understand how the abnormal protein disrupts normal bone growth and development," said Hecht, associate professor in the UT-Houston Medical School division of medical genetics. Pseudoachondroplasia is a condition marked by disproportionate short stature. Bowing or knock-knee deformities of the legs are common in childhood and often require surgical correction. In adulthood, all pseudoachondroplastic dwarves suffer from osteoarthritis and approximately 50 percent require hip replacement. "Basically, their joints wear out early. The hips go first, followed by the knees, ankles, shoulders and elbows," said Hecht. "It's very painful." Hecht's research was funded by a grant from Shriners Hospital for Crippled Children, headquartered in Tampa, Florida. Other researchers participating in this project include investigators from The University of Texas-Houston Medical School, the National Center for Human Genome Research at the National Institutes of Health, the Human Genome Center at Lawrence Livermore National Laboratory, and Harvard Medical School. April 1997 Violence linked to gene defect - A gene linked to pleasure-seeking behavior and addictions also may play a role in murder and violence, new research suggests. This pleasure gene normally is involved in the flow of dopamine, a powerful brain chemical that provides people with their sense of well-being. When defective, the gene diminishes dopamine function, driving a person to drink, take drugs or engage in other activities that give dopamine a boost. Studies suggest people who have a variant of the gene, called DRD2 A1 allele, are prone to becoming smokers, violent alcoholics, gambling addicts and drug addicts. "We think they're seeking out ways of fixing the lack of pleasure," says Kenneth Blum, University of Texas Health Science Center, San Antonio. "You might be a pleasure seeker for alcohol, drugs, sex or maybe you get it from violence or murder." In a study presented Wednesday at an American Psychiatric Association meeting, Blum and 17 researchers found the DRD2 A1 allele in: Six of 11 children in a residential treatment program for pathological violence but only one of 30 normal children. Half of 109 people diagnosed with "schizoid/avoidant" personality. They are introverted, withdrawn, emotionally cold and distant. Other research by psychoanalyst Anneliese Pontius, Harvard Medical School, has tied schizoid/avoidant disorder to more than a dozen bizarre murders. She is testing her subjects for the DRD2 A1 variant. If the findings are confirmed, Blum says problem children could be screened for the gene and given drugs, special diets and other treatments known to boost dopamine. August 1997 Californian scientists say they can control the light given off by a green fluorescent protein derived from a jellyfish (Nature, 1997, 388, 355). The protein molecule produces yellow light when irradiated by a certain wavelength of light but then darkens after a while. However, another wavelength will switch the glow back on. The protein could be useful as light markers in biological processes or in optical storage devices. A retired SmithKline Beecham executive is to form a new biotechnology company called Adprotech based on some of SB's protein patents and lab equipment. SB will take a 10% stake in the company. Japan has decided to support controls on the international trade in genetically modified products such microorganisms, tomatoes and grapeseed oil. Canada is to host a meeting in October to negotiate a protocol on trading such products under the 1992 Convention on Biological Diversity. September 1997 Two scientists have gone on trial in Boston in what is believed to be the first biotechnology espionage case. The two, who were arrested in 1994, are accused of trying to sell erythroprotein, a genetically engineered growth hormone, to the KGB. One of the men had helped develop the treatment at Integrated Genetics, which is now owned by Genzyme. A six-year project to map the entire genetic sequence of the ubiquitous bacterium Escherichia Coli has been completed. The 4.6 million base sequence will be a useful tool for genetics, molecular biology, biotechnology and medicine. (Science, 1997, 277, 1453).