SSL2, a suppressor of a stem-loop mutation in the HIS4 leader encodes the yeast homolog of human ERCC-3.

Reversion of haploid, His4- yeast containing a stem-loop mutation in the 5' UTR that blocks HIS4 translation initiation identified four unlinked suppressor genes, SSL1-SSL4, which restore His4+ expression. The SSL2 gene encodes an essential 95 kd protein with ATP-dependent helicase motifs. SSL2 protein is 54% identical to the protein encoded by the human gene, ERCC-3, for which a defective form causes xeroderma pigmentosum and Cockayne's syndrome. An SSL2 allele made to resemble the defective ERCC-3 gene confers UV light hypersensitivity to yeast cells. Hence, SSL2 is the functional homolog of ERCC-3. However, the SSL2 suppressor gene does not restore HIS4 expression by removal of the stem-loop from DNA or the mRNA. We propose that SSL2 and ERCC-3 may have two functions, one defined by a UV repair defect, and a second essential function that is related to gene expression.