IKAP/Elp1 is required in vivo for neurogenesis and neuronal survival, but not for neural crest migration.

ABSTRACT: Familial Dysautonomia (FD; Hereditary Sensory Autonomic Neuropathy; HSAN III) manifests from a failure in development of the peripheral sensory and autonomic nervous systems. The disease results from a point mutation in the IKBKAP gene, which encodes the IKAP protein, whose function is still unresolved in the developing nervous system. Since the neurons most severely depleted in the disease derive from the neural crest, and in light of data identifying a role for IKAP in cell motility and migration, it has been suggested that FD results from a disruption in neural crest migration. To determine the function of IKAP during development of the nervous system, we (1) first determined the spatial-temporal pattern of IKAP expression in the developing peripheral nervous system, from the onset of n

SUBMITTER: Hunnicutt BJ 

PROVIDER: S-EPMC3285659 | biostudies-literature | 2012

REPOSITORIES: biostudies-literature