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© 2026 CatalystPrivacy TermsReference data under the licences listed on the attribution page.

Proteins/UNIPROT:Q9UJA2

CRLS1

Protein·UNIPROT:Q9UJA2Cardiolipin synthase

Open in the graph

Catalyzes the synthesis of cardiolipin (CL) (diphosphatidylglycerol) by specifically transferring a phosphatidyl group from CDP-diacylglycerol to phosphatidylglycerol (PG). CL is a key phospholipid in mitochondrial membranes and plays important roles in maintaining the functional integrity and dynamics of mitochondria under both optimal and stress conditions..

Function from UniProtKB (Q9UJA2), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

Inferred

Inferred — not reported by a source. Evidence key

GenCC submitters' classifications

2 rows

combined oxidative phosphorylation deficiency 57 has a germline basis in CRLS1DiseaseGenCC HGNC:16148|MONDO:0859337Inferred only
mitochondrial disease has a germline basis in CRLS1DiseaseGenCC HGNC:16148|MONDO:0044970Inferred only

Statements

49 statements

49 statements · 6 reference databases · Subject: CRLS1 · Sources: Bgee, Hetionet, Reactome, Rhea, UniProt, WikiPathways

Search this listOrder

CRLS1 covaries with GARIN6ProteinHetionet hetionet:covaries:196472-54675Inferred only
CRLS1 covaries with JOSD2ProteinHetionet hetionet:covaries:126119-54675Inferred only
CRLS1 covaries with MPV17L2ProteinHetionet hetionet:covaries:54675-84769Inferred only
PASD1 covaries with CRLS1ProteinHetionet hetionet:covaries:139135-54675Inferred only
CRLS1 covaries with WASHC2AProteinHetionet hetionet:covaries:387680-54675Inferred only
CRLS1 is expressed in adipose tissueAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in adrenal glandAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in adult mammalian kidneyAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in apex of heartAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in body of pancreasAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in body of stomachAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in cortex of kidneyAnatomyBgee ENSG00000088766Inferred only

Show the remaining 37

CRLS1 is expressed in duodenumAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in endometriumAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in fundus of stomachAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in gastrocnemiusAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in heartAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in heart left ventricleAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in hindlimb stylopod muscleAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in left adrenal glandAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in left adrenal gland cortexAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in liverAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in mucosa of transverse colonAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in pancreasAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in rectumAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in right adrenal glandAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in right adrenal gland cortexAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in right atrium auricular regionAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in right lobe of liverAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in right lungAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in Skeletal muscle tissueAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in stomachAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in subcutaneous adipose tissueAnatomyBgee ENSG00000088766Inferred only
CRLS1 is expressed in upper lobe of left lungAnatomyBgee ENSG00000088766Inferred only
MCM6 interacts with CRLS1ProteinHetionet hetionet:interacts:4175-54675Inferred only
MCM7 interacts with CRLS1ProteinHetionet hetionet:interacts:4176-54675Inferred only
UBC interacts with CRLS1ProteinHetionet hetionet:interacts:54675-7316Inferred only
CRLS1 participates in Acyl chain remodelling of PGPathwayReactome R-HSA-1482925Inferred only
CRLS1 participates in Glycerolipids and glycerophospholipidsPathwayWikiPathways WP4722Inferred only
CRLS1 participates in Glycerophospholipid biosynthesisPathwayReactome R-HSA-1483206Inferred only
CRLS1 participates in Glycerophospholipid biosynthetic pathwayPathwayWikiPathways WP2533Inferred only
CRLS1 participates in MetabolismPathwayReactome R-HSA-1430728Inferred only
CRLS1 participates in Metabolism of lipidsPathwayReactome R-HSA-556833Inferred only
CRLS1 participates in Phospholipid metabolismPathwayReactome R-HSA-1483257Inferred only
CRLS1 participates in Synthesis of CLPathwayReactome R-HSA-1483076Inferred only
CRLS1 requires the cofactor divalent metal cationCompoundUniProt Q9UJA2Inferred only
a 1,2-diacyl-sn-glycero-3-phospho-(1'-sn-glycerol) is a substrate of CRLS1CompoundRhea RHEA:32931Inferred only
a cardiolipin is a substrate of CRLS1CompoundRhea RHEA:32931Inferred only
a CDP-1,2-diacyl-sn-glycerol is a substrate of CRLS1CompoundRhea RHEA:32931Inferred only

Reactions

2 reactions

MAM01426m+MAM02715ma cardiolipin+cytidine 5'-monophosphate+proton
Catalysed by CRLS1
Reaction pageLeft to rightBalance undeterminableEC 2.7.8.-mitochondrial matrix
Human-GEM MAR00586CC BY 4.0Reference data
a CDP-1,2-diacyl-sn-glycerol+a 1,2-diacyl-sn-glycero-3-phospho-(1'-sn-glycerol)a cardiolipin+CMP+hydron
Catalysed by CRLS1

Other names

6 names

Resolves to: CRLS1

Also called: C20orf155CLSCLS1CMP-formingGCD10 homologProtein GCD10 homolog

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Direction not asserted

Balance undeterminable

Rhea RHEA:32931CC BY 4.0Reference data