LETM1

Structure

The LETM1 protein has a transmembrane domain and a casein kinase 2 and protein kinase C phosphorylation site. The LETM1 gene is expressed in the mitochondria of many eukaryotes indicating that this is a conserved mitochondrial protein.

Function

LETM1 is a eukaryotic protein that is expressed in the inner membrane of mitochondria. Experiments performed with human cells have been interpreted to indicate that it functions as a component of a Ca²⁺/H⁺ antiporter. Experimental results with yeast cells have been interpreted as suggesting that LETM1 functions as a component of a K⁺/H⁺ antiporter. The Drosophila melanogaster LETM1 protein has been shown to functionally substitute for the K⁺/H⁺ antiporter function in yeast cells.

Clinical significance

Deletion of LETM1 is thought to be involved in the development of Wolf–Hirschhorn syndrome in humans.