InterPro domain: IPR011685

This is a group of mainly eukaryotic proteins are inner mitochondrial membrane proteins which play a role in potassium and hydrogen ion exchange [ 1 , 2 ]. Some features found in LETM1, such as a transmembrane domain and a CK2 and PKC phosphorylation site [ 3 ], are relatively conserved throughout the family. Deletion of LETM1 is thought to be involved in the development of Wolf-Hirschhorn syndrome in humans [ 4 ].

1. Electroneutral K+/H+ exchange in mitochondrial membrane vesicles involves Yol027/Letm1 proteins. Biochim. Biophys. Acta 1711, 41-8
2. Identification of nuclear genes encoding mitochondrial proteins: isolation of a collection of D. melanogaster cDNAs homologous to sequences in the Human Gene Index database. Mol. Gen. Genet. 261, 64-70
3. 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