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Protein

Cardiolipin synthase (CMP-forming)

Gene

CRLS1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

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.2 Publications

Catalytic activityi

A CDP-diacylglycerol + a phosphatidylglycerol = a cardiolipin + CMP.1 Publication

Cofactori

a divalent metal cation1 Publication

pH dependencei

Optimum pH is 8.2.1 Publication

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Transferase

Keywords - Biological processi

Lipid biosynthesis, Lipid metabolism, Phospholipid biosynthesis, Phospholipid metabolism

Enzyme and pathway databases

BRENDAi2.7.8.B12. 2681.
ReactomeiR-HSA-1482925. Acyl chain remodelling of PG.
R-HSA-1483076. Synthesis of CL.

Chemistry

SwissLipidsiSLP:000000125.

Names & Taxonomyi

Protein namesi
Recommended name:
Cardiolipin synthase (CMP-forming) (EC:2.7.8.411 Publication)
Short name:
CLS
Alternative name(s):
Protein GCD10 homolog
Gene namesi
Name:CRLS1
Synonyms:C20orf155, CLS1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 20

Organism-specific databases

HGNCiHGNC:16148. CRLS1.

Subcellular locationi

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transmembranei109 – 12921HelicalSequence analysisAdd
BLAST
Transmembranei133 – 15321HelicalSequence analysisAdd
BLAST
Transmembranei190 – 21223HelicalSequence analysisAdd
BLAST
Transmembranei250 – 27021HelicalSequence analysisAdd
BLAST
Transmembranei272 – 29221HelicalSequence analysisAdd
BLAST

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA25697.

Polymorphism and mutation databases

BioMutaiCRLS1.
DMDMi37537857.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 301301Cardiolipin synthase (CMP-forming)PRO_0000056816Add
BLAST

Proteomic databases

MaxQBiQ9UJA2.
PaxDbiQ9UJA2.
PeptideAtlasiQ9UJA2.
PRIDEiQ9UJA2.

PTM databases

iPTMnetiQ9UJA2.
PhosphoSiteiQ9UJA2.

Expressioni

Tissue specificityi

Highly expressed in tissues such as heart, skeletal muscle and liver.1 Publication

Gene expression databases

BgeeiENSG00000088766.
CleanExiHS_CRLS1.
ExpressionAtlasiQ9UJA2. baseline and differential.
GenevisibleiQ9UJA2. HS.

Organism-specific databases

HPAiCAB069896.

Interactioni

Protein-protein interaction databases

BioGridi120096. 2 interactions.
STRINGi9606.ENSP00000368140.

Structurei

3D structure databases

ProteinModelPortaliQ9UJA2.
SMRiQ9UJA2. Positions 109-186.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG1617. Eukaryota.
COG0558. LUCA.
GeneTreeiENSGT00390000001607.
HOGENOMiHOG000010898.
HOVERGENiHBG051203.
InParanoidiQ9UJA2.
KOiK08744.
OMAiSRYFNPC.
OrthoDBiEOG091G0DIC.
PhylomeDBiQ9UJA2.
TreeFamiTF314169.

Family and domain databases

InterProiIPR000462. CDP-OH_P_trans.
[Graphical view]
PfamiPF01066. CDP-OH_P_transf. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q9UJA2-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MLALRVARGS WGALRGAAWA PGTRPSKRRA CWALLPPVPC CLGCLAERWR
60 70 80 90 100
LRPAALGLRL PGIGQRNHCS GAGKAAPRPA AGAGAAAEAP GGQWGPASTP
110 120 130 140 150
SLYENPWTIP NMLSMTRIGL APVLGYLIIE EDFNIALGVF ALAGLTDLLD
160 170 180 190 200
GFIARNWANQ RSALGSALDP LADKILISIL YVSLTYADLI PVPLTYMIIS
210 220 230 240 250
RDVMLIAAVF YVRYRTLPTP RTLAKYFNPC YATARLKPTF ISKVNTAVQL
260 270 280 290 300
ILVAASLAAP VFNYADSIYL QILWCFTAFT TAASAYSYYH YGRKTVQVIK

D
Length:301
Mass (Da):32,593
Last modified:May 1, 2000 - v1
Checksum:i15CD406D29D3C405
GO
Isoform 2 (identifier: Q9UJA2-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-99: Missing.
     100-102: PSL → MPQ

Note: No experimental confirmation available.
Show »
Length:202
Mass (Da):22,584
Checksum:iA7B6D9B4DEE662B4
GO

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei1 – 9999Missing in isoform 2. CuratedVSP_041398Add
BLAST
Alternative sequencei100 – 1023PSL → MPQ in isoform 2. CuratedVSP_041399

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
DQ386730 mRNA. Translation: ABD46888.1.
AF241784 mRNA. Translation: AAG44472.1.
AL035461 Genomic DNA. Translation: CAB55278.1.
CH471133 Genomic DNA. Translation: EAX10401.1.
CH471133 Genomic DNA. Translation: EAX10402.1.
AL832419 mRNA. Translation: CAH10649.1.
CCDSiCCDS13096.1. [Q9UJA2-1]
CCDS46578.1. [Q9UJA2-2]
RefSeqiNP_001120930.1. NM_001127458.1. [Q9UJA2-2]
NP_001310490.1. NM_001323561.1.
NP_001310491.1. NM_001323562.1.
NP_001310492.1. NM_001323563.1.
NP_001310493.1. NM_001323564.1.
NP_061968.1. NM_019095.5. [Q9UJA2-1]
UniGeneiHs.743956.

Genome annotation databases

EnsembliENST00000378863; ENSP00000368140; ENSG00000088766. [Q9UJA2-1]
ENST00000378868; ENSP00000368145; ENSG00000088766. [Q9UJA2-2]
GeneIDi54675.
KEGGihsa:54675.
UCSCiuc002wmn.5. human. [Q9UJA2-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
DQ386730 mRNA. Translation: ABD46888.1.
AF241784 mRNA. Translation: AAG44472.1.
AL035461 Genomic DNA. Translation: CAB55278.1.
CH471133 Genomic DNA. Translation: EAX10401.1.
CH471133 Genomic DNA. Translation: EAX10402.1.
AL832419 mRNA. Translation: CAH10649.1.
CCDSiCCDS13096.1. [Q9UJA2-1]
CCDS46578.1. [Q9UJA2-2]
RefSeqiNP_001120930.1. NM_001127458.1. [Q9UJA2-2]
NP_001310490.1. NM_001323561.1.
NP_001310491.1. NM_001323562.1.
NP_001310492.1. NM_001323563.1.
NP_001310493.1. NM_001323564.1.
NP_061968.1. NM_019095.5. [Q9UJA2-1]
UniGeneiHs.743956.

3D structure databases

ProteinModelPortaliQ9UJA2.
SMRiQ9UJA2. Positions 109-186.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi120096. 2 interactions.
STRINGi9606.ENSP00000368140.

Chemistry

SwissLipidsiSLP:000000125.

PTM databases

iPTMnetiQ9UJA2.
PhosphoSiteiQ9UJA2.

Polymorphism and mutation databases

BioMutaiCRLS1.
DMDMi37537857.

Proteomic databases

MaxQBiQ9UJA2.
PaxDbiQ9UJA2.
PeptideAtlasiQ9UJA2.
PRIDEiQ9UJA2.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000378863; ENSP00000368140; ENSG00000088766. [Q9UJA2-1]
ENST00000378868; ENSP00000368145; ENSG00000088766. [Q9UJA2-2]
GeneIDi54675.
KEGGihsa:54675.
UCSCiuc002wmn.5. human. [Q9UJA2-1]

Organism-specific databases

CTDi54675.
GeneCardsiCRLS1.
HGNCiHGNC:16148. CRLS1.
HPAiCAB069896.
MIMi608188. gene.
neXtProtiNX_Q9UJA2.
PharmGKBiPA25697.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1617. Eukaryota.
COG0558. LUCA.
GeneTreeiENSGT00390000001607.
HOGENOMiHOG000010898.
HOVERGENiHBG051203.
InParanoidiQ9UJA2.
KOiK08744.
OMAiSRYFNPC.
OrthoDBiEOG091G0DIC.
PhylomeDBiQ9UJA2.
TreeFamiTF314169.

Enzyme and pathway databases

BRENDAi2.7.8.B12. 2681.
ReactomeiR-HSA-1482925. Acyl chain remodelling of PG.
R-HSA-1483076. Synthesis of CL.

Miscellaneous databases

ChiTaRSiCRLS1. human.
GenomeRNAii54675.
PROiQ9UJA2.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000088766.
CleanExiHS_CRLS1.
ExpressionAtlasiQ9UJA2. baseline and differential.
GenevisibleiQ9UJA2. HS.

Family and domain databases

InterProiIPR000462. CDP-OH_P_trans.
[Graphical view]
PfamiPF01066. CDP-OH_P_transf. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiCRLS1_HUMAN
AccessioniPrimary (citable) accession number: Q9UJA2
Secondary accession number(s): D3DW09
, E9PAT4, Q27RP0, Q69YQ5
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 3, 2003
Last sequence update: May 1, 2000
Last modified: September 7, 2016
This is version 121 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 20
    Human chromosome 20: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.