Preface II

The two papers that follow represent work within the Neurobiology Research Theme of the University of Kansas Medical Center. The research programs of the Center recognize that mental retardation is not only a biological condition but also a behavioral and societal condition requiring interdisciplinary collaboration from several flelds for effective progress. Accordingly, our research themes include a mix of behavioral and biological projects that focus on areas such as language and communication disorders, intervention and treatment strategies, risk factors, neurobiological processes and cellular and molecular aspects of early embryonic development. Our Center’s neurobiology research theme emphasizes basic research and includes work on animal models of brain pathology and disease, neuronal plasticity and mechanisms of brain injury. Emphasis is on interdisciplinary approaches aimed at understanding not only the relevant cellular and molecular events but also the manner in which these events alter the organization and function of brain systems underlying motor, sensory and cognitive behaviors. The paper by Dr Fechtel and colleagues that follows describes work on a promising rodent model of spina bifida that should enable examination of cellular and molecular processes underlying the failure of neural tube closure. Neural tube defects are among the most common of birth defects; their prevalence world wide ranges up to lO/l,OOO live births. In addition, neural tube defects contribute greatly to perinatal loss. For the first time, the work from our Center on this model holds the promise of testing fundamental molecular hypotheses about the origin neural tube defects. The paper by Dr Levine and colleagues presents some of their recent findings on the twitcher mouse model of globoid cell leukodystrophy (Krabbe’s disease). Globoid cell leukodystrophy is a demyelinating disease of infancy caused by deficiency of the enzyme galactocerebroside Beta-galactosidase. The disease is characterized by progressive spasticity and blindness and is invariably fatal. One of the unique aspects of this study is the application of Fourier Transform Infrared Microspectroscopy to measure the distribution of a specific chemical (psychosine) throughout the brain. This is a powerful approach that not only provides information about the content of specific chemical substances in the brain but also provides this information with high spatial resolution never before possible.