LETM1

LETM1 is a protein that is localized to the inner mitochondrial membrane. The protein functions to maintain the mitochondrial tubular shapes and is required for normal mitochondrial morphology and cellular viability. Mutations in this gene lead to Wolf-Hirschhorn syndrome, a complex malformation syndrome caused by the deletion of parts of the distal short arm of chromosome 4. Related pseudogenes have been identified on chromosomes 8, 15 and 19.

leucine zipper and EF-hand containing transmembrane protein 1

Mitochondrial proton/calcium antiporter that mediates proton-dependent calcium efflux from mitochondrion (PubMed:19797662).
Crucial for the maintenance of mitochondrial tubular networks and for the assembly of the supercomplexes of the respiratory chain (PubMed:18628306).
Required for the maintenance of the tubular shape and cristae organization (PubMed:18628306).
In contrast to SLC8B1/NCLX, does not constitute the major factor for mitochondrial calcium extrusion (PubMed:24898248).

Calcium export from the mitochondrionManual Assertion Based On ExperimentIDA:UniProtKB
Cellular metal ion homeostasisManual Assertion Based On ExperimentIBA:GO_Central
Cristae formationManual Assertion Based On ExperimentIMP:UniProtKB
Mitochondrial calcium ion homeostasisManual Assertion Based On ExperimentIDA:UniProtKB
Mitochondrial calcium ion transmembrane transportManual Assertion Based On ExperimentIDA:UniProtKB
Negative regulation of mitochondrial calcium ion concentrationManual Assertion Based On ExperimentIMP:UniProtKB
Protein hexamerizationISS:UniProtKB
Protein homooligomerizationISS:UniProtKB
Regulation of cellular hyperosmotic salinity responseManual Assertion Based On ExperimentIMP:UniProtKB

Mitochondrion inner membrane

Mitochondrial intermembrane: 116-208
Helical: 209-229
Mitochondrial matrix: 230-739