CAENORHABDITIS-ELEGANS CDNA FOR A MENKES WILSON-DISEASE GENE HOMOLOG AND ITS FUNCTION IN A YEAST CCC2 GENE DELETION MUTANT/

The full-length cDNA coding for a putative copper transporting P-type ATPase (Cu2+-ATPase) was cloned from Caenorhabditis elegans, The putat ive Cu2+-ATPase is a 1,238-amino acid protein, and highly homologous t o the Menkes and Wilson disease gene products mutations of which are r esponsible for human defects of copper metabolism, The Saccharomyces c erevisiae mutant with a disrupted CCC2 gene (yeast Menkes/Wilson disea se gene homologue) was rescued by the cDNA for the C. elegans Cu2+-ATP ase but not by the cDNA with an Asp-786 (an invariant phosphorylation site) to Asn mutation, suggesting that the C. elegans Cu2+-ATPase func tions as a copper transporter in yeast, The expressed C. elegans prote in was detected in yeast vacuolar membranes by immunofluorescence micr oscopy. The yeast expression system may facilitate further studies on copper transporting P-type ATPases.