InterPro domain: IPR044202

This family represents a group of LETM1-like mitochondrial inner membrane proteins, including LETM1/LETM2 from mammals and YLH47/MDM38 from yeasts

LETM1 is a proton/calcium antiporter that mediates proton-dependent calcium efflux from mitochondrion [ 1 ]. LETM2 is preferentially expressed in testis and sperm. In humans, deletion of LETM1 causes Wolf-Hirschhorn syndrome [ 2 ].

MDM38 is involved in potassium homeostasis [ 3 ] and, with MBA1, it plays a role in ribosomal translation and protein insertion into the inner membrane [ 4 ].

1. Genome-wide RNAi screen identifies Letm1 as a mitochondrial Ca2+/H+ antiporter. Science 326, 144-7
2. LETM1, a novel gene encoding a putative EF-hand Ca(2+)-binding protein, flanks the Wolf-Hirschhorn syndrome (WHS) critical region and is deleted in most WHS patients. Genomics 60, 218-25
3. The LETM1/YOL027 gene family encodes a factor of the mitochondrial K+ homeostasis with a potential role in the Wolf-Hirschhorn syndrome. J. Biol. Chem. 279, 30307-15
4. Ribosome-binding proteins Mdm38 and Mba1 display overlapping functions for regulation of mitochondrial translation. Mol. Biol. Cell 21, 1937-44