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Proteins/UNIPROT:O95202

LETM1

Protein·UNIPROT:O95202Mitochondrial proton/calcium exchanger protein2 compounds

Open in the graph

Plays an important role in maintenance of mitochondrial morphology and in mediating either calcium or potassium/proton antiport. Mediates proton-dependent calcium efflux from mitochondrion. Also functions as an electroneutral mitochondrial proton/potassium exchanger. Crucial for the maintenance of mitochondrial tubular networks and for the assembly of the supercomplexes of the respiratory chain. Required for the maintenance of the tubular shape and cristae organization..

Function from UniProtKB (O95202), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 2 · transported by

Inferred

Assembled routes, not reported.

What routes through it

2 compounds

2 compounds.

0 reported routes2 assembled routes

Ca(2+)ElementCHEBI:29108Inferred only40 paths40 endpoints
K(+)

What the reference databases state

GenCC submitters' classifications

1 row

neurodegeneration, childhood-onset, with multisystem involvement due to mitochondrial dysfunction has a germline basis in LETM1DiseaseGenCC HGNC:6556|MONDO:0859304Inferred only

Statements

61 statements

61 statements · 7 reference databases · Subject: LETM1 · Sources: Bgee, gene ontology, Hetionet, Human Protein Atlas, Reactome, Rhea, WikiPathways

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LETM1 covaries with ZNF831ProteinHetionet hetionet:covaries:128611-3954Inferred only
LETM1 is expressed in adipose tissueAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in adrenal glandAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in Ammon's hornAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in bone marrowAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in breastAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in bronchusAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in caudate nucleusAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in cerebellumAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in cerebral cortexAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in colonAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in duodenumAnatomyHuman Protein Atlas ENSG00000168924Inferred only

Show the remaining 49

LETM1 is expressed in endometriumAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in esophagusAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in fallopian tubeAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in gallbladderAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in liverAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in lungAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in lymph nodeAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in mouth mucosaAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in mucosa of transverse colonAnatomyBgee ENSG00000168924Inferred only
LETM1 is expressed in myocardiumAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in nasopharynxAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in pancreasAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in parathyroid glandAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in rectumAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in saliva-secreting glandAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in seminal vesicleAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in Skeletal muscle tissueAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in Small intestineAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in spleenAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in stomachAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in testisAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in thyroid glandAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in tonsilAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in urinary bladderAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in uterine cervixAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in vaginaAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in vermiform appendixAnatomyHuman Protein Atlas ENSG00000168924Inferred only
LETM1 is expressed in zone of skinAnatomyHuman Protein Atlas ENSG00000168924Inferred only
BCS1L interacts with LETM1ProteinHetionet hetionet:interacts:3954-617Inferred only
MRPL58 interacts with LETM1ProteinHetionet hetionet:interacts:3396-3954Inferred only
LETM1 interacts with UBCProteinHetionet hetionet:interacts:3954-7316Inferred only
LETM1 participates in 4p16 3 copy number variationPathwayWikiPathways WP5365Inferred only
LETM1 participates in Aerobic respiration and respiratory electron transportPathwayReactome R-HSA-1428517Inferred only
LETM1 participates in Complex III assemblyPathwayReactome R-HSA-9865881Inferred only
LETM1 participates in MetabolismPathwayReactome R-HSA-1430728Inferred only
LETM1 participates in Mitochondrial calcium ion transportPathwayReactome R-HSA-8949215Inferred only
LETM1 participates in Respiratory electron transportPathwayReactome R-HSA-611105Inferred only
LETM1 participates in RHO GTPase cyclePathwayReactome R-HSA-9012999Inferred only
LETM1 participates in RHOG GTPase cyclePathwayReactome R-HSA-9013408Inferred only
LETM1 participates in Signal TransductionPathwayReactome R-HSA-162582Inferred only
LETM1 participates in Signaling by Rho GTPasesPathwayReactome R-HSA-194315Inferred only
LETM1 participates in Signaling by Rho GTPases, Miro GTPases and RHOBTB3PathwayReactome R-HSA-9716542Inferred only
LETM1 participates in Transport of small moleculesPathwayReactome R-HSA-382551Inferred only
LETM1 regulates negative regulation of mitochondrial calcium ion concentrationBiological processgene ontology O95202|GO:0051562Inferred only
LETM1 regulates regulation of cellular hyperosmotic salinity responseBiological processgene ontology O95202|GO:1900069Inferred only
Ca(2+) is transported by LETM1CompoundRhea RHEA:72199Inferred only
hydron is transported by LETM1CompoundRhea RHEA:72199Inferred only
hydron is transported by LETM1CompoundRhea RHEA:29467Inferred only
K(+) is transported by LETM1CompoundRhea RHEA:29467Inferred only

Reactions

2 reactions

K(+)+hydronK(+)+hydron
Transported by LETM1 or SLC9A9 or SLC9A6 or SLC9A7 or GHITM or SLC17A7 or SLC17A6
Reaction pageLeft to rightBalanced
Rhea RHEA:29467CC BY 4.0Reference data
Ca(2+)+2hydronCa(2+)

Other names

3 names

Resolves to: LETM1

Also called: Electroneutral mitochondrial K(+)/H(+)exchangerKHELeucine zipper-EF-hand-containing transmembrane protein 1

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Element

·CHEBI:29103

Inferred only40 paths40 endpoints

+

Transported by LETM1 or GHITM

Reaction pageDirection not assertedBalanced

Rhea RHEA:72199CC BY 4.0Reference data