Ontology highlight
ABSTRACT:
SUBMITTER: Genabai NK
PROVIDER: S-EPMC5557895 | biostudies-other | 2017 Aug
REPOSITORIES: biostudies-other
Scientific reports 20170815 1
Spinal muscular atrophy (SMA) is caused by the low levels of survival motor neuron (SMN) protein and is characterized by motor neuron degeneration and muscle atrophy. Respiratory failure causes death in SMA but the underlying molecular mechanism is unknown. The zinc finger protein ZPR1 interacts with SMN. ZPR1 is down regulated in SMA patients. We report that ZPR1 functions downstream of SMN to regulate HoxA5 levels in phrenic motor neurons that control respiration. Spatiotemporal inactivation o ...[more]