This report describes Drosophila models of copper metabolism disorder (postulated), SLC31A-related. Either excess amounts of or deficiencies of elemental copper result in cellular damage and disease. There are three Drosophila genes, Ctr1A, Ctr1B and Ctr1C, orthologous to the human copper transporter genes SLC31A1 and SLC31A2. In flies, overexpression of the Dmel\Ctr1B has been used to model copper toxicity. In combination with Dmel\ATP7 (see FBhh0000438), RNAi-effected reduction in the expression of Dmel\Ctr1A has been used to model copper deficiency.
A UAS construct of the Hsap\SLC31A1 gene has been introduced into flies. Heterologous rescue (functional complementation) has been demonstrated for a lethal allele of Dmel\Ctr1A.
Pan-neuronal overexpression of Dmel\Ctr1B results in a reduction in viability; these effects are exacerbated by dietary copper supplementation and rescued by copper limitation, indicating a copper toxicity phenotype. This phenotype mimics Wilson disease (MIM:277900, FBhh0000079).
[updated Nov. 2016 by FlyBase; FBrf0222196]
Many to many: 2 human genes to 3 Drosophila genes; the other gene in human is SLC31A1.
Many to many: 2 human genes to 3 Drosophila genes; the other gene in human is SLC31A2.
High-scoring ortholog of human gene SLC31A1; moderate-scoring ortholog of human SLC31A2 (3 Drosophila to 2 human). Dmel\Ctr1A is the most broadly expressed of the 3 fly genes. It shares 34-40% identity and 54-55% similarity with the human genes.
Low- to moderate-scoring ortholog of human genes SLC31A1 and SLC31A2 (3 Drosophila to 2 human). Dmel\Ctr1B is the most highly expressed in larval and adult digestive system and fat body. It shares 26-38% identity and 47-54% similarity with the human genes.
