Bringing CNS Members Together to Make Children’s Lives Better


Elliott Sheer, MD, PhD

Elliott Sheer, MD, PhD

Elliott Sherr attended Stanford University and was awarded B.A.S in Philosophy and Biology in 1984. Studies under the auspices of the NIH Medical Science Training Program at Columbia University were undertaken in 1987 and 1995. His scientific investigations under the direction of Dr. Lloyd Greene concerned aspects of neuronal growth cone motility.

Dr. Sherr identified and characterized a novel family of small myosin proteins that have subsequently been shown to perform central functions in vesicular trafficking and lamellipodial dynamics. His M.D. and Ph.D. degrees were awarded in 1995. At the time of his graduation he also received the prestigious Helen M. Sciarra Award in Neurology and the Miriam Berkman Spotnitz Award in Research. He was Visiting Scientist, Catholic University, Santiago, Chile prior to initiating formal training in pediatrics, neurology, and child neurology at the University of California,

San Francisco in 1997. In 1999 he received the UCSF Department of Pediatrics Outstanding Clinical Fellow Award. During his training, Dr. Sherr’s broad range of interests and competencies included particular interest in epilepsy and developmental disturbances.

Upon completion of training in 2000 he received a five-year NIH Neurological Sciences Academic Development Award, serving for the first two years as Adjunct Instructor in Neurology and Pediatrics. He was also named, upon completion of his formal training, Co-Director of the UCSF Pediatric Neurometabolic/Neurogenetics Program and Clinic. Dr. Sherr joined the laboratory of Dr. David Bredt in the UCSF Department of Physiology in order to investigate basic mechanisms of epileptogenesis. His investigations employing a murine model of audiogenic seizures resulted in the cloning of a mutated gene, jams1. He has recently demonstrated the effects this mutation may have on a novel system of signaling cascades in synaptic proteins of the brains of these mice. Perhaps even more importantly, he has discerned that vulnerability to these conditioned seizures is developmentally regulated by additional genes at loci distinct from the jams1 gene. Thus, he is poised to investigate a problem that is critical to child neurology: the factors that determine developmental vulnerability to seizures and epilepsy. In 2004 he competed successfully for a two year Child Neurology Foundation Scientific Award, exciting work that may have profound importance.

Dr. Sherr has also devoted attention to investigations concerning agenesis of the corpus callosum (ACC), based upon clinical interest in the disabilities experienced by such patients, such as epilepsy, autism, motor and intellectual limitations. In a cohort of 25 patients with this disorder, he was able to identify three with chromosome deletions and duplications in regions 2q37, 6qter, and 8p. He is poised to greatly expand his cadre both from within his own clinics and in collaboration with the California Birth Defects Monitoring Program, with a database containing information on more than 100,000 children. Moreover, Dr. Sherr has organized and is carrying out important work in collaboration with A. J. Barkovich that combines assessment of gross morphology by MRI with degree of disruption of white matter pathways assessed by high angular resolution diffusion imaging to subclassify ACC for ensuing neurodevelopmental testing and molecular biological investigations.

The work of Dr. Sherr and his colleagues is very important. ACC, a relatively common finding that is readily identified, often in infancy, by standard CT or MRI imaging is, in fact, a family of disorders, the individual subgroups of which have not as yet been very usefully defined by gross morphology. The lack of a sensitive and reliable subclassification of ACC is a hindrance to the identification of the genetic basis for particular subgroups which would, in turn, permit bench investigations of the developmental mechanisms of particular groupings of observed disorders. Such basic information will likely enlarge understanding of mechanisms of neurodevelopmental diseases. For the individual child and family dealing with ACC, refinement of classification on the basis of advanced imaging and genetic studies will permit ensuing longitudinal studies with both observant clinical monitoring and standardized neurodevelopmental testing. This will not only provide more sensitive information upon which more accurate early prognostic statements may be based. It will also permit the particular coping strategies of individuals and their families to be noted and appreciated and it will quite likely provide a basis upon which targeted remediations may be developed and implemented, remediations that may in turn result in improvements of outcome that will agreeably revise initial prognostic estimates.

Dr. Sherr’s work has been supported by funding from the NINDS(KO2) and the March of Dimes; further research efforts concerning this important topic are the subject of the research proposal for which he has been awarded the 2006 Philip R. Dodge Young Investigator Award. Since 2001 Dr. Sherr has held the position of Investigator at the UCSF-Stanford Lysosomal Disease Center. In 2002 he was promoted to Assistant Adjunct Professor in the UCSF Departments of Neurology and Pediatrics. In 2005 he was named Director, Brain Development Research Program, UCSF.

Dr. Sherr is not only a clear thinker, but a clear communicator of ideas, reflected in his research presentations at national meetings and his excellent publications in major journals. He has characteristically conveyed in both scientific and clinical realms infectious enthusiasm and commitment that derives in considerable degree from his overriding interest in the welfare of children. His particular interests and abilities have permitted him to expand the activities in the Neurometabolic Clinic where his powers of observation and diagnostic acumen serve him and his patients well. Not content with the recognition and confirmation of known genetic disorders that may be found in the minority of patients presenting to such a clinic, Dr. Sherr has a passion for the difficult job of identifying heretofore unknown genetic explanations in the remaining patients. His track record suggests that this work will provide more than the small dignity of a particularizing name for a disorder affecting a child and that child’s family, thus providing the opportunity of prognostic refinement as well as the other possible cascade of benefits from the study and classification of genetic diseases noted above.

Dr. Sherr is located in just the right place within a rich network of collaborative colleagues and all other facilities necessary to bring phase after phase of his exciting work to fruition. Dr. Sherr’s busy and productive laboratory activities have not prevented him from continued engagement in the clinic, achieving a balance wherein each form of concentration informs the other and provides him with the energizing effects of change of activity. A third form of apparently preferred activity is teaching, and, to the considerable enjoyment and enlightenment of students, residents, and colleagues, he represents – in an era where such an achievement has become rare – a genuine “triple threat.” His achievement is more than just managing to keep abreast of the rapid changes in medicine and science. He is an individual whose concern and compassion and whose appreciation of the individuality of child or parent coping with disease represents another important form of teaching that can only be accomplished by example.

The work Dr. Sherr is undertaking could, in addition to the other important things it may accomplish, provide an important advance in that most difficult of all objectives, arriving at some understanding the manner in which thought and behavior are modulated by the human brain.