The present invention includes an isolated nucleic acid comprising a nucleic acid sequence encoding a SMN&Dgr7 degron and the encoded polypeptide. The invention also includes inhibitors of SMN&Dgr7 degron. The invention also includes compositions and methods for mitigating SMN deficiency by targeting inhibition of factors that mediate SMN&Dgr7-degron dependent degradation of SMN&Dgr7.