Characterization of a novel human putative mitochondrial transporter homologous to the yeast mitochondrial RNA splicing proteins 3 and 4

We report here a novel human gene, hMRS314, encoding a putative mitochondrial transporter structurally and functionally homologous to the,,east mitochondrial RNA splicing proteins 3 and 4, These proteins belong to the family of mitochondrial carrier proteins (MCF) and are likely tb function as solute carriers. hMRS314 spans similar to 10 kb of genomic DNA on chromosome 10q24 and consists of four exons that encode a 364-aa protein with six transmembrane domains. A putative splice variant, encoding a 177-aa protein with three transmembrane domains, was also identified. hMRS3/4 has a well-conserved signature sequence of R ICF and is targeted into the mitochondria, When expressed in yeast, hMRS3/4 efficiently restores the mitochondrial functions in mrs3 degrees mrs4 degrees knock-out mutants. Ubiquitous expression in human tissues and a well-conserved structure and function suggest an important role for hMRS3/4 in human cells. (C) 2001 Published by Elsevier Science B,V, on behalf of the Federation of European Biochemical Societies.