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Protein

ATP-dependent zinc metalloprotease YME1L1

Gene

Yme1l1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

ATP-dependent metalloprotease that catalyzes the degradation of folded and unfolded proteins with a suitable degron sequence in the mitochondrial intermembrane region (By similarity). Plays an important role in regulating mitochondrial morphology and function by cleaving OPA1 at position S2, giving rise to a form of OPA1 that promotes maintenance of normal mitochondrial structure (PubMed:17709429, PubMed:24616225, PubMed:26785494, PubMed:27495975). Ensures cell proliferation, maintains normal cristae morphology and complex I respiration activity, promotes antiapoptotic activity and protects mitochondria from the accumulation of oxidatively damaged membrane proteins (By similarity). Required for normal, constitutive degradation of PRELID1 (PubMed:26785494). Catalyzes the degradation of OMA1 in response to membrane depolarization. Required to control the accumulation of nonassembled respiratory chain subunits (NDUFB6, OX4 and ND1) (By similarity).By similarity4 Publications

Cofactori

Zn2+By similarityNote: Binds 1 zinc ion per subunit.By similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi541Zinc; catalyticBy similarity1
Active sitei542By similarity1
Metal bindingi545Zinc; catalyticBy similarity1
Metal bindingi619Zinc; catalyticUniRule annotation1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi321 – 328ATPSequence analysis8

GO - Molecular functioni

  • ATP binding Source: UniProtKB-KW
  • ATP-dependent peptidase activity Source: UniProtKB
  • metal ion binding Source: UniProtKB-KW
  • metalloendopeptidase activity Source: InterPro

GO - Biological processi

Keywordsi

Molecular functionHydrolase, Metalloprotease, Protease
LigandATP-binding, Metal-binding, Nucleotide-binding, Zinc

Enzyme and pathway databases

ReactomeiR-MMU-8949664 Processing of SMDT1

Names & Taxonomyi

Protein namesi
Recommended name:
ATP-dependent zinc metalloprotease YME1L1 (EC:3.4.24.-1 Publication)
Alternative name(s):
ATP-dependent metalloprotease FtsH1
YME1-like protein 1
Gene namesi
Name:Yme1l1
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 2

Organism-specific databases

MGIiMGI:1351651 Yme1l1

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 237Mitochondrial matrixSequence analysisAdd BLAST237
Transmembranei238 – 258HelicalSequence analysisAdd BLAST21
Topological domaini259 – 715Mitochondrial intermembraneSequence analysisAdd BLAST457

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Disruption phenotypei

Complete embryonic lethality; embryos present a clear developmental delay at 8.5 dpc, and the hearts of mutant embryos fail to beat properly at 9.5 and 10.5 dpc. Cardiomyocyte-specific gene disruption gives rise to animals that develop dilated cardiomyopathy and myocardial fibrosis at about 20 weeks after birth; mutants have a median life span of about 46 weeks, much shorter than wild-type. Mitochondria from mutant cardiomyocytes are smaller than normal, but have normal cristae architecture and display no significant difference in the assembly of respiratory complexes. Keeping mice with a cardiomyocyte-specific gene disruption on a high-fat diet leads to weight gain and reduced glucose tolerance, and prevents the development of cardiomyopathy. Mice with Yme1l gene disruption in cardiomyocytes and skeletal muscle have a median life span of 125 weeks, similar to wild-type. Their heart function is normal, in spite of the presence of fragmented mitochondria due to the loss of Opa1 cleavage at position S2. Skeletal muscle mitochondrial dysfunction is known to be associated with impaired insulin signaling and glucose intolerance, and as expected, these mice display impaired glucose homeostasis with decreased fasting insulin levels in the blood serum and glucose intolerance. Mice with a double, cardiomyocyte-specific gene disruption of Yme1l and Oma1 have normal cardiac function and do not display myocardial fibrosis. Likewise, cardiomyocyte mitochondria have normal morphology. Mice with a skeletal muscle Yme1l gene disruption plus a double, cardiomyocyte-specific gene disruption of Yme1l and Oma1 display normal glucose tolerance.1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000846681 – 715ATP-dependent zinc metalloprotease YME1L1Add BLAST715

Post-translational modificationi

Proteolytically processed by mitochondrial processing peptidase (MPP) to generate the mature form.By similarity

Proteomic databases

EPDiO88967
MaxQBiO88967
PaxDbiO88967
PeptideAtlasiO88967
PRIDEiO88967

PTM databases

iPTMnetiO88967
PhosphoSitePlusiO88967

Expressioni

Tissue specificityi

Detected in heart and skeletal muscle (at protein level).1 Publication

Gene expression databases

BgeeiENSMUSG00000026775 Expressed in 289 organ(s), highest expression level in pineal body
CleanExiMM_YME1L1
GenevisibleiO88967 MM

Interactioni

Subunit structurei

Homohexamer; may also form heterohexamers. Exists in several complexes of 600-1100 kDa. Interacts with AFG1L.By similarity

Protein-protein interaction databases

BioGridi205184, 2 interactors
IntActiO88967, 1 interactor
STRINGi10090.ENSMUSP00000028117

Structurei

3D structure databases

ProteinModelPortaliO88967
SMRiO88967
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

In the N-terminal section; belongs to the AAA ATPase family.Curated
In the C-terminal section; belongs to the peptidase M41 family.Curated

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0734 Eukaryota
COG0465 LUCA
GeneTreeiENSGT00550000074836
HOGENOMiHOG000217276
HOVERGENiHBG057127
InParanoidiO88967
KOiK08955
OMAiCLYRQHS
OrthoDBiEOG091G02Y9
PhylomeDBiO88967
TreeFamiTF105005

Family and domain databases

HAMAPiMF_01458 FtsH, 1 hit
InterProiView protein in InterPro
IPR003593 AAA+_ATPase
IPR003959 ATPase_AAA_core
IPR003960 ATPase_AAA_CS
IPR005936 FtsH
IPR027417 P-loop_NTPase
IPR000642 Peptidase_M41
IPR037219 Peptidase_M41-like
PfamiView protein in Pfam
PF00004 AAA, 1 hit
PF01434 Peptidase_M41, 1 hit
SMARTiView protein in SMART
SM00382 AAA, 1 hit
SUPFAMiSSF140990 SSF140990, 1 hit
SSF52540 SSF52540, 1 hit
TIGRFAMsiTIGR01241 FtsH_fam, 1 hit
PROSITEiView protein in PROSITE
PS00674 AAA, 1 hit

Sequencei

Sequence statusi: Complete.

O88967-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MFSLSSTVQP QVTIPLSHLI NAFHSPKNIS VSVNTPVSQK QHRDTVPEHE
60 70 80 90 100
APSSEPVLNL RDLGLSELKI GQIDKMVENL LPGFYKDKRV SSCWHTSHIS
110 120 130 140 150
AQSFFENKYG HLDMFSTLRS SSLYRQHPKT LRSICSDLQY FPVFIQSRGF
160 170 180 190 200
KTLKSRTRRL QSTSERLVEA QNIAPSFVKG FLLRDRGTDL ESLDKLMKTK
210 220 230 240 250
NIPEAHQDAF KTGFAEGFLK AQALTQKTND SLRRTRLILF VLLLFGIYGL
260 270 280 290 300
LKNPFLSVRF RTTTGLDSAV DPVQMKNVTF EHVKGVEEAK QELQEVVEFL
310 320 330 340 350
KNPQKFTVLG GKLPKGILLV GPPGTGKTLL ARAVAGEADV PFYYASGSEF
360 370 380 390 400
DEMFVGVGAS RIRNLFREAK ANAPCVIFID ELDSVGGKRI ESPMHPYSRQ
410 420 430 440 450
TINQLLAEMD GFKPNEGVII IGATNFPEAL DNALIRPGRF DMQVTVPRPD
460 470 480 490 500
VKGRTEILKW YLNKIKFDKS VDPEIIARGT VGFSGAELEN LVNQAALKAA
510 520 530 540 550
VDGKEMVTMK ELEFSKDKIL MGPERRSVEI DNKNKTITAY HESGHAIIAY
560 570 580 590 600
YTKDAMPINK ATIMPRGPTL GHVSLLPEND RWNETRAQLL AQMDVSMGGR
610 620 630 640 650
VAEELIFGTD HITTGASSDF DNATKIAKRM VTKFGMSEKL GVMTYSDTGK
660 670 680 690 700
LSPETQSAIE QEIRILLRES YERAKHILKT HAKEHKNLAE ALLTYETLDA
710
KEIQIVLEGK KLEVR
Length:715
Mass (Da):80,028
Last modified:November 1, 1998 - v1
Checksum:i28A861C55B305106
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF090430 mRNA Translation: AAC35558.1
AY136286 mRNA Translation: AAN17724.1
AY136287 mRNA Translation: AAN17725.1
AK079175 mRNA Translation: BAC37568.1
BC007128 mRNA Translation: AAH07128.1
CCDSiCCDS15728.1
RefSeqiNP_038799.1, NM_013771.5
UniGeneiMm.23335

Genome annotation databases

EnsembliENSMUST00000028117; ENSMUSP00000028117; ENSMUSG00000026775
GeneIDi27377
KEGGimmu:27377
UCSCiuc008ioe.2 mouse

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF090430 mRNA Translation: AAC35558.1
AY136286 mRNA Translation: AAN17724.1
AY136287 mRNA Translation: AAN17725.1
AK079175 mRNA Translation: BAC37568.1
BC007128 mRNA Translation: AAH07128.1
CCDSiCCDS15728.1
RefSeqiNP_038799.1, NM_013771.5
UniGeneiMm.23335

3D structure databases

ProteinModelPortaliO88967
SMRiO88967
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi205184, 2 interactors
IntActiO88967, 1 interactor
STRINGi10090.ENSMUSP00000028117

PTM databases

iPTMnetiO88967
PhosphoSitePlusiO88967

Proteomic databases

EPDiO88967
MaxQBiO88967
PaxDbiO88967
PeptideAtlasiO88967
PRIDEiO88967

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000028117; ENSMUSP00000028117; ENSMUSG00000026775
GeneIDi27377
KEGGimmu:27377
UCSCiuc008ioe.2 mouse

Organism-specific databases

CTDi10730
MGIiMGI:1351651 Yme1l1

Phylogenomic databases

eggNOGiKOG0734 Eukaryota
COG0465 LUCA
GeneTreeiENSGT00550000074836
HOGENOMiHOG000217276
HOVERGENiHBG057127
InParanoidiO88967
KOiK08955
OMAiCLYRQHS
OrthoDBiEOG091G02Y9
PhylomeDBiO88967
TreeFamiTF105005

Enzyme and pathway databases

ReactomeiR-MMU-8949664 Processing of SMDT1

Miscellaneous databases

PROiPR:O88967
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000026775 Expressed in 289 organ(s), highest expression level in pineal body
CleanExiMM_YME1L1
GenevisibleiO88967 MM

Family and domain databases

HAMAPiMF_01458 FtsH, 1 hit
InterProiView protein in InterPro
IPR003593 AAA+_ATPase
IPR003959 ATPase_AAA_core
IPR003960 ATPase_AAA_CS
IPR005936 FtsH
IPR027417 P-loop_NTPase
IPR000642 Peptidase_M41
IPR037219 Peptidase_M41-like
PfamiView protein in Pfam
PF00004 AAA, 1 hit
PF01434 Peptidase_M41, 1 hit
SMARTiView protein in SMART
SM00382 AAA, 1 hit
SUPFAMiSSF140990 SSF140990, 1 hit
SSF52540 SSF52540, 1 hit
TIGRFAMsiTIGR01241 FtsH_fam, 1 hit
PROSITEiView protein in PROSITE
PS00674 AAA, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiYMEL1_MOUSE
AccessioniPrimary (citable) accession number: O88967
Secondary accession number(s): Q7TNN5
Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 13, 2004
Last sequence update: November 1, 1998
Last modified: November 7, 2018
This is version 151 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Peptidase families
    Classification of peptidase families and list of entries
  3. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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