RNA binding proteins in health and disease

Abstract

Studies of the molecular genetics for Amyotrophic lateral sclerosis (ALS) and other motor neuron disorders have thrust a class of proteins, termed RNA binding proteins (RBPs), into the limelight. A striking number of mutations linked to motor neuron diseases occur in RBPs. The reason appears to derive from the unique structures and biology of RBP, as well as the unique abundance of these proteins in the extra-nuclear domains of neurons. The material discussed in this chapter will explicate the biology of RBPs, provide insight into how this contributes to the pathophysiology of neurodegenerative diseases, and also speculate on potential roles of RNA binding proteins in the inflammatory response.