[HTML][HTML] Ubiquitin-specific protease 9x deubiquitinates and stabilizes the spinal muscular atrophy protein-survival motor neuron
KJ Han, DG Foster, NY Zhang, K Kanisha… - Journal of Biological …, 2012 - ASBMB
Spinal muscular atrophy (SMA), the leading genetic disorder of infant mortality, is caused by
low levels of survival motor neuron (SMN) protein. Currently it is not clear how the SMN
protein levels are regulated at the post-transcriptional level. In this report, we find that Usp9x,
a deubiquitinating enzyme, stably associates with the SMN complex via directly interacting
with SMN. Usp9x deubiquitinates SMN that is mostly mono-and di-ubiquitinated. Knockdown
of Usp9x promotes SMN degradation and reduces the protein levels of SMN and the SMN …
low levels of survival motor neuron (SMN) protein. Currently it is not clear how the SMN
protein levels are regulated at the post-transcriptional level. In this report, we find that Usp9x,
a deubiquitinating enzyme, stably associates with the SMN complex via directly interacting
with SMN. Usp9x deubiquitinates SMN that is mostly mono-and di-ubiquitinated. Knockdown
of Usp9x promotes SMN degradation and reduces the protein levels of SMN and the SMN …