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Protein

LETM1 and EF-hand domain-containing protein 1, mitochondrial

Gene

LETM1

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

Functioni

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.1 Publication

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Calcium bindingi676 – 688PROSITE-ProRule annotationAdd BLAST13

GO - Molecular functioni

  • calcium ion binding Source: ProtInc
  • ribosome binding Source: InterPro

GO - Biological processi

  • cellular metal ion homeostasis Source: GO_Central
  • cristae formation Source: UniProtKB
Complete GO annotation...

Keywords - Ligandi

Calcium, Metal-binding

Enzyme and pathway databases

BioCyciZFISH:ENSG00000168924-MONOMER.

Protein family/group databases

TCDBi2.A.97.1.1. the mitochondrial inner membrane k(+)/h(+) and ca(2+)/h(+) exchanger (letm1) family.

Names & Taxonomyi

Protein namesi
Recommended name:
LETM1 and EF-hand domain-containing protein 1, mitochondrial
Alternative name(s):
Leucine zipper-EF-hand-containing transmembrane protein 1
Gene namesi
Name:LETM1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 4

Organism-specific databases

HGNCiHGNC:6556. LETM1.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini116 – 208Mitochondrial intermembraneSequence analysisAdd BLAST93
Transmembranei209 – 229HelicalSequence analysisAdd BLAST21
Topological domaini230 – 739Mitochondrial matrixSequence analysisAdd BLAST510

GO - Cellular componenti

  • integral component of membrane Source: UniProtKB-KW
  • mitochondrial inner membrane Source: UniProtKB
  • mitochondrion Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi3954.
OpenTargetsiENSG00000168924.
Orphaneti280. Wolf-Hirschhorn syndrome.
PharmGKBiPA30335.

Polymorphism and mutation databases

BioMutaiLETM1.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 115MitochondrionSequence analysisAdd BLAST115
ChainiPRO_0000017694116 – 739LETM1 and EF-hand domain-containing protein 1, mitochondrialAdd BLAST624

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei597N6-acetyllysineCombined sources1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiO95202.
MaxQBiO95202.
PaxDbiO95202.
PeptideAtlasiO95202.
PRIDEiO95202.

PTM databases

iPTMnetiO95202.
PhosphoSitePlusiO95202.
SwissPalmiO95202.

Expressioni

Gene expression databases

BgeeiENSG00000168924.
CleanExiHS_LETM1.
GenevisibleiO95202. HS.

Organism-specific databases

HPAiHPA011029.
HPA011100.

Interactioni

Subunit structurei

Can form 2 complexes: a major (300 kDa) and a minor complex (500-600 kDa). Interacts with BCS1L.1 Publication

Binary interactionsi

WithEntry#Exp.IntActNotes
PSMA3P257883EBI-1052895,EBI-348380

Protein-protein interaction databases

BioGridi110145. 22 interactors.
IntActiO95202. 10 interactors.
MINTiMINT-3002664.
STRINGi9606.ENSP00000305653.

Structurei

3D structure databases

ProteinModelPortaliO95202.
SMRiO95202.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini252 – 537Letm1 RBDPROSITE-ProRule annotationAdd BLAST286
Domaini663 – 698EF-handPROSITE-ProRule annotationAdd BLAST36

Coiled coil

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Coiled coili115 – 136Sequence analysisAdd BLAST22
Coiled coili462 – 490Sequence analysisAdd BLAST29
Coiled coili537 – 627Sequence analysisAdd BLAST91
Coiled coili708 – 739Sequence analysisAdd BLAST32

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi18 – 21Poly-Pro4

Sequence similaritiesi

Contains 1 EF-hand domain.PROSITE-ProRule annotation
Contains 1 Letm1 RBD (ribosome-binding) domain.PROSITE-ProRule annotation

Keywords - Domaini

Coiled coil, Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG1043. Eukaryota.
ENOG410XRSP. LUCA.
GeneTreeiENSGT00390000011225.
HOGENOMiHOG000290246.
HOVERGENiHBG052322.
InParanoidiO95202.
KOiK17800.
OMAiQLEMDQQ.
OrthoDBiEOG091G034T.
PhylomeDBiO95202.
TreeFamiTF316321.

Family and domain databases

Gene3Di1.10.238.10. 1 hit.
InterProiIPR011992. EF-hand-dom_pair.
IPR018247. EF_Hand_1_Ca_BS.
IPR002048. EF_hand_dom.
IPR011685. LETM1.
IPR033122. LETM1_RBD.
[Graphical view]
PfamiPF07766. LETM1. 1 hit.
[Graphical view]
SUPFAMiSSF47473. SSF47473. 1 hit.
PROSITEiPS00018. EF_HAND_1. 1 hit.
PS50222. EF_HAND_2. 1 hit.
PS51758. LETM1_RBD. 1 hit.
[Graphical view]

Sequences (3)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: O95202-1) [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
MASILLRSCR GRAPARLPPP PRYTVPRGSP GDPAHLSCAS TLGLRNCLNV
60 70 80 90 100
PFGCCTPIHP VYTSSRGDHL GCWALRPECL RIVSRAPWTS TSVGFVAVGP
110 120 130 140 150
QCLPVRGWHS SRPVRDDSVV EKSLKSLKDK NKKLEEGGPV YSPPAEVVVK
160 170 180 190 200
KSLGQRVLDE LKHYYHGFRL LWIDTKIAAR MLWRILNGHS LTRRERRQFL
210 220 230 240 250
RICADLFRLV PFLVFVVVPF MEFLLPVAVK LFPNMLPSTF ETQSLKEERL
260 270 280 290 300
KKELRVKLEL AKFLQDTIEE MALKNKAAKG SATKDFSVFF QKIRETGERP
310 320 330 340 350
SNEEIMRFSK LFEDELTLDN LTRPQLVALC KLLELQSIGT NNFLRFQLTM
360 370 380 390 400
RLRSIKADDK LIAEEGVDSL NVKELQAACR ARGMRALGVT EDRLRGQLKQ
410 420 430 440 450
WLDLHLHQEI PTSLLILSRA MYLPDTLSPA DQLKSTLQTL PEIVAKEAQV
460 470 480 490 500
KVAEVEGEQV DNKAKLEATL QEEAAIQQEH REKELQKRSE VAKDFEPERV
510 520 530 540 550
VAAPQRPGTE PQPEMPDTVL QSETLKDTAP VLEGLKEEEI TKEEIDILSD
560 570 580 590 600
ACSKLQEQKK SLTKEKEELE LLKEDVQDYS EDLQEIKKEL SKTGEEKYVE
610 620 630 640 650
ESKASKRLTK RVQQMIGQID GLISQLEMDQ QAGKLAPANG MPTGENVISV
660 670 680 690 700
AELINAMKQV KHIPESKLTS LAAALDENKD GKVNIDDLVK VIELVDKEDV
710 720 730
HISTSQVAEI VATLEKEEKV EEKEKAKEKA EKEVAEVKS
Length:739
Mass (Da):83,354
Last modified:May 1, 1999 - v1
Checksum:i942E9138F299D94F
GO
Isoform 2 (identifier: O95202-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-48: MASILLRSCRGRAPARLPPPPRYTVPRGSPGDPAHLSCASTLGLRNCL → MRHTWPFR
     292-294: KIR → QVL
     295-739: Missing.

Note: May be due to intron retention. No experimental confirmation available.
Show »
Length:254
Mass (Da):29,291
Checksum:i5510CB4D93FFDDDE
GO
Isoform 3 (identifier: O95202-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     292-294: KIR → QVL
     295-739: Missing.

Note: May be due to intron retention.
Show »
Length:294
Mass (Da):33,245
Checksum:i5C8B98ECA4D20E42
GO

Sequence cautioni

The sequence CAB63769 differs from that shown. Reason: Erroneous translation. Incomplete prediction of CDS at the C-terminus.Curated

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0378141 – 48MASIL…LRNCL → MRHTWPFR in isoform 2. 2 PublicationsAdd BLAST48
Alternative sequenceiVSP_037815292 – 294KIR → QVL in isoform 2 and isoform 3. 2 Publications3
Alternative sequenceiVSP_037816295 – 739Missing in isoform 2 and isoform 3. 2 PublicationsAdd BLAST445

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF061025 mRNA. Translation: AAD13138.1.
AK293572 mRNA. Translation: BAG57043.1.
AK310563 mRNA. No translation available.
AL133650 mRNA. Translation: CAB63769.2. Sequence problems.
BC014500 mRNA. Translation: AAH14500.1.
BC021208 mRNA. Translation: AAH21208.1.
CCDSiCCDS3355.1. [O95202-1]
PIRiT43494.
RefSeqiNP_036450.1. NM_012318.2. [O95202-1]
UniGeneiHs.120165.

Genome annotation databases

EnsembliENST00000302787; ENSP00000305653; ENSG00000168924. [O95202-1]
GeneIDi3954.
KEGGihsa:3954.
UCSCiuc003gdv.3. human. [O95202-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF061025 mRNA. Translation: AAD13138.1.
AK293572 mRNA. Translation: BAG57043.1.
AK310563 mRNA. No translation available.
AL133650 mRNA. Translation: CAB63769.2. Sequence problems.
BC014500 mRNA. Translation: AAH14500.1.
BC021208 mRNA. Translation: AAH21208.1.
CCDSiCCDS3355.1. [O95202-1]
PIRiT43494.
RefSeqiNP_036450.1. NM_012318.2. [O95202-1]
UniGeneiHs.120165.

3D structure databases

ProteinModelPortaliO95202.
SMRiO95202.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110145. 22 interactors.
IntActiO95202. 10 interactors.
MINTiMINT-3002664.
STRINGi9606.ENSP00000305653.

Protein family/group databases

TCDBi2.A.97.1.1. the mitochondrial inner membrane k(+)/h(+) and ca(2+)/h(+) exchanger (letm1) family.

PTM databases

iPTMnetiO95202.
PhosphoSitePlusiO95202.
SwissPalmiO95202.

Polymorphism and mutation databases

BioMutaiLETM1.

Proteomic databases

EPDiO95202.
MaxQBiO95202.
PaxDbiO95202.
PeptideAtlasiO95202.
PRIDEiO95202.

Protocols and materials databases

DNASUi3954.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000302787; ENSP00000305653; ENSG00000168924. [O95202-1]
GeneIDi3954.
KEGGihsa:3954.
UCSCiuc003gdv.3. human. [O95202-1]

Organism-specific databases

CTDi3954.
DisGeNETi3954.
GeneCardsiLETM1.
HGNCiHGNC:6556. LETM1.
HPAiHPA011029.
HPA011100.
MIMi604407. gene.
neXtProtiNX_O95202.
OpenTargetsiENSG00000168924.
Orphaneti280. Wolf-Hirschhorn syndrome.
PharmGKBiPA30335.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1043. Eukaryota.
ENOG410XRSP. LUCA.
GeneTreeiENSGT00390000011225.
HOGENOMiHOG000290246.
HOVERGENiHBG052322.
InParanoidiO95202.
KOiK17800.
OMAiQLEMDQQ.
OrthoDBiEOG091G034T.
PhylomeDBiO95202.
TreeFamiTF316321.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000168924-MONOMER.

Miscellaneous databases

ChiTaRSiLETM1. human.
GeneWikiiLETM1.
GenomeRNAii3954.
PROiO95202.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000168924.
CleanExiHS_LETM1.
GenevisibleiO95202. HS.

Family and domain databases

Gene3Di1.10.238.10. 1 hit.
InterProiIPR011992. EF-hand-dom_pair.
IPR018247. EF_Hand_1_Ca_BS.
IPR002048. EF_hand_dom.
IPR011685. LETM1.
IPR033122. LETM1_RBD.
[Graphical view]
PfamiPF07766. LETM1. 1 hit.
[Graphical view]
SUPFAMiSSF47473. SSF47473. 1 hit.
PROSITEiPS00018. EF_HAND_1. 1 hit.
PS50222. EF_HAND_2. 1 hit.
PS51758. LETM1_RBD. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiLETM1_HUMAN
AccessioniPrimary (citable) accession number: O95202
Secondary accession number(s): B4DED2, Q9UF65
Entry historyi
Integrated into UniProtKB/Swiss-Prot: April 12, 2005
Last sequence update: May 1, 1999
Last modified: November 30, 2016
This is version 141 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 4
    Human chromosome 4: 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.