Degradation of survival motor neuron (SMN) protein is mediated via the ubiquitin/proteasome pathway

HC Chang, WC Hung, YJ Chuang, YJ Jong - Neurochemistry international, 2004 - Elsevier
Homozygous deletion or mutation in the survival motor neuron (SMN) 1 gene causes
proximal spinal muscular atrophy (SMA), whereas SMN2 acts as a modifying gene that can
influence the severity of SMA. It has been suggested that restoration of the SMN protein level
in neuronal cells may prevent cell loss and may be helpful for treatment of SMA. Recent
studies indicate that the ubiquitin/proteasome pathway is a major system for proteolysis of
intracellular proteins. In this study, we investigate whether SMN protein is degraded via the …