The human family of deafness/dystonia peptide (DDP) related mitochondrial import proteins

The gene responsible for the human genetic neurodegenerative disorder DFN-1 /MTS encodes a small protein known as deafness/dystonia peptide (DDP), It b ears a strong resemblance to a recently characterized set of zinc-binding y east proteins (Tim8p, Tim9p, Tim10p, Tim12p, and Tim13p) that are implicate d in the import of a class of transmembrane carrier proteins from the cytop lasm to the mitochondrial inner membrane. We describe here the human comple ment of DDP/Tim-like proteins and establish the likely orthologous relation ships between sequences from human, yeast, and other organisms, We also des cribe the expression patterns and chromosomal locations of their genes, whi ch are candidate loci for autosomal recessive neurodegenerative disorders. (C) 1999 Academic Press.