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 mitochondri al transporter structurally and functionally homologous to the,,east mitoch ondrial RNA splicing proteins 3 and 4, These proteins belong to the family of mitochondrial carrier proteins (MCF) and are likely tb function as solut e carriers. hMRS314 spans similar to 10 kb of genomic DNA on chromosome 10q 24 and consists of four exons that encode a 364-aa protein with six transme mbrane domains. A putative splice variant, encoding a 177-aa protein with t hree transmembrane domains, was also identified. hMRS3/4 has a well-conserv ed signature sequence of R ICF and is targeted into the mitochondria, When expressed in yeast, hMRS3/4 efficiently restores the mitochondrial function s in mrs3 degrees mrs4 degrees knock-out mutants. Ubiquitous expression in human tissues and a well-conserved structure and function suggest an import ant role for hMRS3/4 in human cells. (C) 2001 Published by Elsevier Science B,V, on behalf of the Federation of European Biochemical Societies.