Maria Dolores (LOLA) Ledesma, Ph.D.
Center of Molecular Biology in Spain. Molecular Neuobiology Specialty.
Our laboratory is interested in understanding the role that lipids play in neuronal physiology and pathology with special emphasis on its participation in the synapse. In recent years much progress has been made in understanding the protein machinery that regulates synaptic transmission. However, much less is known about the contribution of lipid although essential synaptic activity is regulated by protein binding to the membranes , the lipids are major components . Also, dynamic membranes is the basis for the transmission of information . The fact that most lipidosis caused cognitive defects and mental retardation is also a reflection of the importance of the lipids in this process . Lipids are focused our studies sphingolipids and cholesterol because, among other reasons , they are particularly abundant in neurons , and are capable of forming signaling platforms . Mice were used as experimental models in which enzymes involved in the metabolism of these lipids are genetically altered and which allow in vivo analysis . The models that we are currently mice deficient in acid sphingomyelinase and the Seladin one responsible recycling of sphingomyelin and cholesterol synthesis , respectively. These mice mimic human genetic diseases such as Niemann Pick type A or desmosteroslosis that cause severe mental retardation. Furthermore, we have found lipid abnormalities in these mice ( Crameri et al , EMBO J , 2006,. . . . Galvan et al , Mol Biol Cell, 2008 ) are similar to those found in aged brains. This opens the possibility that our results have implications not only in the formation and maintenance of synapses but also in functional decline during aging. We hope therefore contribute to understanding neurological diseases of childhood and neurodegenerative as Alzheimer ‘s disease.