A subset of SMN complex members have a specific role in tissue regeneration via ERBB pathway-mediated proliferation.

Pei, Wuhong; Xu, Lisha; Chen, Zelin; Slevin, Claire C; Pettie, Kade P; Wincovitch, Stephen; NISC Comparative Sequencing Program; Burgess, Shawn M · NPJ Regen Med · 2020

basic_science · Level V

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Abstract

Spinal muscular atrophy (SMA) is the most common genetic disease in children. SMA is generally caused by mutations in the gene <i>SMN1</i>. The survival of motor neurons (SMN) complex consists of SMN1, Gemins (2-8), and Strap/Unrip. We previously demonstrated <i>smn1</i> and <i>gemin5</i> inhibited tissue regeneration in zebrafish. Here we investigated each individual SMN complex member and identified <i>gemin3</i> as another regeneration-essential gene. These three genes are likely pan-regenerative, since they affect the regeneration of hair cells, liver, and caudal fin. RNA-Seq analysis reveals that <i>smn1</i>, <i>gemin3</i>, and <i>gemin5</i> are linked to a common set of genetic pathways, including the tp53 and ErbB pathways. Additional studies indicated all three genes facilitate regeneration by inhibiting the ErbB pathway, thereby allowing cell proliferation in the injured neuromasts. This study provides a new understanding of the SMN complex and a potential etiology for SMA and potentially other rare unidentified genetic diseases with similar symptoms.