%0 Journal Article
%T From neurodevelopment to neurodegeneration: the interaction of neurofibromin and valosin-containing protein/p97 in regulation of dendritic spine formation
%A Yi-Ping Hsueh
%J Journal of Biomedical Science
%D 2012
%I BioMed Central
%R 10.1186/1423-0127-19-33
%X Neurons are highly differentiated cells composed of several specialized subcellular structures, including soma, dendrites, axon, and numerous synapses. All of these structures play specific roles in signal transduction between or within neurons. Besides dendrites and axons, dendritic spines are particularly interesting structures, which are the tiny protrusions (~0.5-1 ¦Ìm in width and ~1-2 ¦Ìm in length) extending from dendrites. The majority of excitatory synapses in the mammalian nervous system are localized at the tips of dendritic spines [1]. Morphology and density of dendritic spines are controlled by genetic program, neuronal activity, and environmental insults. Indeed, defects of neural development leading to alterations of neuronal morphology are associated with many neurological and neuropsychiatric disorders, including mental retardation, learning disability, autism, attention-deficient hyperactivity disorder, and schizophrenia [2,3]. On the other hand, impairment in maintenance of neuronal morphology is also frequently found in neurodegenerative disorders. For instance, synaptic loss has been suggested as a cause of dementia [4-6]. This review will focus on an example showing that neurodegeneration may be relevant with neurodevelopment in terms of regulation of neuronal morphology. It is regarding a recent finding of the interaction between neurofibromin and valosin-containing protein (VCP).Neurofibromin, the protein product of the neurofibromatosis type I (NF1) gene, regulates the formation of dendritic spines [7], which explains at least partially why patients with NF1 suffer from cognitive defects. Recently, we further identified that VCP/p97 interacts with neurofibromin and demonstrated that VCP/p97 also controls dendritic spinogenesis of neurons [8]. Mutations in the VCP gene result in inclusion body myopathy with Paget's disease of bone and frontotemporal dementia (IBMPFD) and amyotrophic lateral sclerosis (ALS). The function of VCP in spinogenesis p
%K Dendritic spine formation
%K IBMPFD
%K Neurodevelopmental disorder
%K Neurofibromatosis Type I
%K neurofibromin
%K statin
%K VCP/p97.
%U http://www.jbiomedsci.com/content/19/1/33