A role for the survival of motor neuron protein in mRNP assembly and transport
Current opinion in neurobiology, 2016•Elsevier
Highlights•Spinal muscular atrophy (SMA) is characterized by axonal and synaptic
defects.•SMN-complexes are actively transported in axons and to neuromuscular junctions
(NMJs) during development.•SMN-deficiency causes defects in the delivery of mRNPs in
motor neuron axons.•SMN acts as chaperone for RNP assembly, regulating diverse RNA
processing pathways.•mRNP assembly defects may contribute to selective motor system
dysfunction in SMA.Localization and local translation of mRNA plays a key role in neuronal …
defects.•SMN-complexes are actively transported in axons and to neuromuscular junctions
(NMJs) during development.•SMN-deficiency causes defects in the delivery of mRNPs in
motor neuron axons.•SMN acts as chaperone for RNP assembly, regulating diverse RNA
processing pathways.•mRNP assembly defects may contribute to selective motor system
dysfunction in SMA.Localization and local translation of mRNA plays a key role in neuronal …
Highlights
- Spinal muscular atrophy (SMA) is characterized by axonal and synaptic defects.
- SMN-complexes are actively transported in axons and to neuromuscular junctions (NMJs) during development.
- SMN-deficiency causes defects in the delivery of mRNPs in motor neuron axons.
- SMN acts as chaperone for RNP assembly, regulating diverse RNA processing pathways.
- mRNP assembly defects may contribute to selective motor system dysfunction in SMA.
Localization and local translation of mRNA plays a key role in neuronal development and function. While studies in various systems have provided insights into molecular mechanisms of mRNA transport and local protein synthesis, the factors that control the assembly of mRNAs and mRNA binding proteins into messenger ribonucleoprotein (mRNP) transport granules remain largely unknown. In this review we will discuss how insights on a motor neuron disease, spinal muscular atrophy (SMA), is advancing our understanding of regulated assembly of transport competent mRNPs and how defects in their assembly and delivery may contribute to the degeneration of motor neurons observed in SMA and other neurological disorders.
Elsevier