UniProtKB - Q3ULF4 (SPG7_MOUSE)
Paraplegin
Spg7
Functioni
ATP-dependent zinc metalloprotease. Plays a role in the formation and regulation of the mitochondrial permeability transition pore (mPTP) and its proteolytic activity is dispensable for this function (By similarity).
By similarityCuratedCaution
Cofactori
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Binding sitei | 312 | ATP; via amide nitrogen and carbonyl oxygenBy similarity | 1 | |
Binding sitei | 492 | ATPBy similarity | 1 | |
Metal bindingi | 574 | Zinc; catalyticBy similarity | 1 | |
Active sitei | 575 | By similarity | 1 | |
Metal bindingi | 578 | Zinc; catalyticBy similarity | 1 | |
Metal bindingi | 650 | Zinc; catalyticBy similarity | 1 |
Regions
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Nucleotide bindingi | 349 – 357 | ATPBy similarity | 9 |
GO - Molecular functioni
- ATP binding Source: UniProtKB-KW
- ATP-dependent peptidase activity Source: InterPro
- ATP hydrolysis activity Source: InterPro
- metalloendopeptidase activity Source: Ensembl
- zinc ion binding Source: InterPro
GO - Biological processi
- anterograde axonal transport Source: MGI
- cell adhesion Source: MGI
- mitochondrial outer membrane permeabilization involved in programmed cell death Source: UniProtKB
- mitochondrial protein processing Source: GO_Central
- mitochondrion organization Source: MGI
- protein-containing complex assembly Source: GO_Central
- regulation of cell adhesion Source: MGI
- regulation of mitochondrial membrane permeability Source: UniProtKB
Keywordsi
Molecular function | Hydrolase, Metalloprotease, Protease |
Ligand | ATP-binding, Metal-binding, Nucleotide-binding, Zinc |
Enzyme and pathway databases
BRENDAi | 3.4.24.B18, 3474 |
Reactomei | R-MMU-8949664, Processing of SMDT1 |
Protein family/group databases
MEROPSi | M41.006 |
Names & Taxonomyi
Protein namesi | Recommended name: Paraplegin1 Publication (EC:3.4.24.-)Alternative name(s): Spastic paraplegia 7 proteinImported |
Gene namesi | Name:Spg7Imported |
Organismi | Mus musculus (Mouse) |
Taxonomic identifieri | 10090 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Myomorpha › Muroidea › Muridae › Murinae › Mus › Mus |
Proteomesi |
|
Organism-specific databases
MGIi | MGI:2385906, Spg7 |
VEuPathDBi | HostDB:ENSMUSG00000000738 |
Subcellular locationi
Mitochondrion
- Mitochondrion inner membrane 2 Publications; Multi-pass membrane protein Sequence analysis
Mitochondrion
- m-AAA complex Source: MGI
- mitochondrial inner membrane Source: UniProtKB
- mitochondrial permeability transition pore complex Source: UniProtKB
- mitochondrion Source: MGI
Other locations
- axon cytoplasm Source: GOC
Topology
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Topological domaini | 106 – 144 | Mitochondrial matrixCuratedAdd BLAST | 39 | |
Transmembranei | 145 – 165 | Helical; Name=1Sequence analysisAdd BLAST | 21 | |
Topological domaini | 166 – 248 | Mitochondrial intermembraneCuratedAdd BLAST | 83 | |
Transmembranei | 249 – 269 | Helical; Name=2Sequence analysisAdd BLAST | 21 | |
Topological domaini | 270 – 781 | Mitochondrial matrixCuratedAdd BLAST | 512 |
Keywords - Cellular componenti
Membrane, Mitochondrion, Mitochondrion inner membranePathology & Biotechi
Disruption phenotypei
Mutagenesis
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Mutagenesisi | 575 | E → Q: Absence of proteolytic activity. No loss of its processing into the mature form. 1 Publication | 1 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transit peptidei | 1 – 43 | Mitochondrion1 PublicationAdd BLAST | 43 | |
PropeptideiPRO_0000442306 | 44 – 105 | Removed in mature form1 PublicationAdd BLAST | 62 | |
ChainiPRO_0000442307 | 106 – 781 | ParapleginAdd BLAST | 676 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 505 | 3'-nitrotyrosineCombined sources | 1 |
Post-translational modificationi
Keywords - PTMi
NitrationProteomic databases
EPDi | Q3ULF4 |
MaxQBi | Q3ULF4 |
PaxDbi | Q3ULF4 |
PRIDEi | Q3ULF4 |
ProteomicsDBi | 261134 |
PTM databases
iPTMneti | Q3ULF4 |
PhosphoSitePlusi | Q3ULF4 |
Expressioni
Tissue specificityi
Gene expression databases
Bgeei | ENSMUSG00000000738, Expressed in retinal neural layer and 239 other tissues |
ExpressionAtlasi | Q3ULF4, baseline and differential |
Genevisiblei | Q3ULF4, MM |
Interactioni
Subunit structurei
Forms heterooligomers with AFG3L1 and AFG3L2 (PubMed:22563492, PubMed:17101804, PubMed:19656850).
Component of the mitochondrial permeability transition pore complex (mPTPC), at least composed of SPG7, VDAC1 and PPIF (By similarity).
Interacts with AFG3L2; the interaction is required for the efficient assembly of mitochondrial complex I (PubMed:22563492, PubMed:19656850).
Interacts with AFG3L1 (PubMed:19656850).
Interacts with MAIP1.
Interacts with VDAC1 and PPIF (By similarity).
By similarity3 PublicationsProtein-protein interaction databases
BioGRIDi | 231586, 3 interactors |
STRINGi | 10090.ENSMUSP00000119552 |
Miscellaneous databases
RNActi | Q3ULF4, protein |
Family & Domainsi
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 22 – 56 | DisorderedSequence analysisAdd BLAST | 35 | |
Regioni | 103 – 135 | DisorderedSequence analysisAdd BLAST | 33 | |
Regioni | 701 – 781 | Interaction with PPIFBy similarityAdd BLAST | 81 |
Compositional bias
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Compositional biasi | 107 – 135 | Basic and acidic residuesSequence analysisAdd BLAST | 29 |
Sequence similaritiesi
Keywords - Domaini
Transit peptide, Transmembrane, Transmembrane helixPhylogenomic databases
eggNOGi | KOG0731, Eukaryota |
GeneTreei | ENSGT00940000156329 |
InParanoidi | Q3ULF4 |
OMAi | TRMKSMK |
OrthoDBi | 217929at2759 |
TreeFami | TF105003 |
Family and domain databases
Gene3Di | 1.20.58.760, 1 hit 3.40.50.300, 1 hit |
HAMAPi | MF_01458, FtsH, 1 hit |
InterProi | View protein in InterPro IPR003593, AAA+_ATPase IPR041569, AAA_lid_3 IPR003959, ATPase_AAA_core IPR005936, FtsH IPR027417, P-loop_NTPase IPR011546, Pept_M41_FtsH_extracell IPR000642, Peptidase_M41 IPR037219, Peptidase_M41-like |
Pfami | View protein in Pfam PF00004, AAA, 1 hit PF17862, AAA_lid_3, 1 hit PF06480, FtsH_ext, 1 hit PF01434, Peptidase_M41, 1 hit |
SMARTi | View protein in SMART SM00382, AAA, 1 hit |
SUPFAMi | SSF140990, SSF140990, 1 hit SSF52540, SSF52540, 1 hit |
TIGRFAMsi | TIGR01241, FtsH_fam, 1 hit |
(1+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry has 1 described isoform and 7 potential isoforms that are computationally mapped.Show allAlign All
10 20 30 40 50
MAAALLLLRG LRPGPEPRPR RLWGLLSGRG PGLSSGAGAR RPYAARGTPV
60 70 80 90 100
GPAAAGGHAP QSLLLRILTP SFEGISGLLL KQHIVPNAVR LWPLSGSTLY
110 120 130 140 150
FNTSRMKQKN KDNDKPKGKT PEDDEEEKRR KEREDQMYRE RLRTLFIIAL
160 170 180 190 200
VMSLLNSLST SGGSISWADF VNEMLAKGEV QRVQVVPESD VVEVYLHPGA
210 220 230 240 250
VVFGRPRLAL MYRMQVANID KFEEKLRAAE DELNIESKDR IPVSYKRTGF
260 270 280 290 300
FGNALYALGM TAVGLAILWY VFRLAGMTGR EGGFSAFNQL KMARFTIVDG
310 320 330 340 350
KTGKGVSFQD VAGMHEAKLE VREFVDYLKS PERFLQLGAK VPKGALLLGP
360 370 380 390 400
PGCGKTLLAK AVATEAQVPF LAMAGPEFVE VIGGLGAARV RSLFKEARAR
410 420 430 440 450
APCIVYIDEI DAVGKKRSTS MSGFSNTEEE QTLNQLLVEM DGMGTTDHVI
460 470 480 490 500
VLASTNRADV LDNALMRPGR LDRHVFIDLP TLQERREIFE QHLKGLKLTQ
510 520 530 540 550
PSSFYSQRLA ELTPGFSGAD IANICNEAAL HAAREGHTSV HTFNFEYAVE
560 570 580 590 600
RVIAGTAKKS KILSKEEQRV VAFHESGHAL VGWLLEHTEA VMKVSIAPRT
610 620 630 640 650
NAALGFSQML PRDQYLFTKE QLFERMCMAL GGRAAEAISF SRVTSGAQDD
660 670 680 690 700
LRKVTRIAYS MVKQFGMAPS IGPVSFPEAQ EGLMGIGRRP FSQGLQQMMD
710 720 730 740 750
HEAKLLVAKA YRHTEKVLLD NLDKLQALAN ALLEKEVINY EDIEALIGPP
760 770 780
PHGPKKMIAP QKWIDAEKER QASGEEEAPA P
Computationally mapped potential isoform sequencesi
There are 7 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketD3YZN4 | D3YZN4_MOUSE | Paraplegin | Spg7 | 676 | Annotation score: | ||
D3YXB7 | D3YXB7_MOUSE | Paraplegin | Spg7 | 744 | Annotation score: | ||
G3UX97 | G3UX97_MOUSE | Paraplegin | Spg7 | 209 | Annotation score: | ||
D3Z342 | D3Z342_MOUSE | Paraplegin | Spg7 | 230 | Annotation score: | ||
F6W695 | F6W695_MOUSE | Paraplegin | Spg7 | 253 | Annotation score: | ||
F6VTG4 | F6VTG4_MOUSE | Paraplegin | Spg7 | 90 | Annotation score: | ||
A0A1I7Q4C2 | A0A1I7Q4C2_MOUSE | Paraplegin | Spg7 | 145 | Annotation score: |
Sequence cautioni
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 165 | I → T in AAO21098 (Ref. 2) Curated | 1 | |
Sequence conflicti | 168 | A → S in AAI38142 (PubMed:19468303).Curated | 1 | |
Sequence conflicti | 310 | D → G in AAO21098 (Ref. 2) Curated | 1 | |
Sequence conflicti | 471 | L → F in AAN03852 (PubMed:14722615).Curated | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AF512565 mRNA Translation: AAN03852.1 AF547215 mRNA Translation: AAO21098.1 AK145540 mRNA Translation: BAE26494.1 AC121819 Genomic DNA No translation available. BC024466 mRNA Translation: AAH24466.1 BC024986 mRNA Translation: AAH24986.1 BC051051 mRNA Translation: AAH51051.1 BC096690 mRNA Translation: AAH96690.1 Different initiation. BC055488 mRNA Translation: AAH55488.1 Different initiation. BC138141 mRNA Translation: AAI38142.1 |
CCDSi | CCDS40508.1 |
RefSeqi | NP_694816.3, NM_153176.4 |
Genome annotation databases
Ensembli | ENSMUST00000149248; ENSMUSP00000119552; ENSMUSG00000000738 |
GeneIDi | 234847 |
KEGGi | mmu:234847 |
UCSCi | uc009nuc.1, mouse |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AF512565 mRNA Translation: AAN03852.1 AF547215 mRNA Translation: AAO21098.1 AK145540 mRNA Translation: BAE26494.1 AC121819 Genomic DNA No translation available. BC024466 mRNA Translation: AAH24466.1 BC024986 mRNA Translation: AAH24986.1 BC051051 mRNA Translation: AAH51051.1 BC096690 mRNA Translation: AAH96690.1 Different initiation. BC055488 mRNA Translation: AAH55488.1 Different initiation. BC138141 mRNA Translation: AAI38142.1 |
CCDSi | CCDS40508.1 |
RefSeqi | NP_694816.3, NM_153176.4 |
3D structure databases
AlphaFoldDBi | Q3ULF4 |
SMRi | Q3ULF4 |
ModBasei | Search... |
Protein-protein interaction databases
BioGRIDi | 231586, 3 interactors |
STRINGi | 10090.ENSMUSP00000119552 |
Protein family/group databases
MEROPSi | M41.006 |
PTM databases
iPTMneti | Q3ULF4 |
PhosphoSitePlusi | Q3ULF4 |
Proteomic databases
EPDi | Q3ULF4 |
MaxQBi | Q3ULF4 |
PaxDbi | Q3ULF4 |
PRIDEi | Q3ULF4 |
ProteomicsDBi | 261134 |
Protocols and materials databases
Antibodypediai | 30863, 376 antibodies from 21 providers |
DNASUi | 234847 |
Genome annotation databases
Ensembli | ENSMUST00000149248; ENSMUSP00000119552; ENSMUSG00000000738 |
GeneIDi | 234847 |
KEGGi | mmu:234847 |
UCSCi | uc009nuc.1, mouse |
Organism-specific databases
CTDi | 6687 |
MGIi | MGI:2385906, Spg7 |
VEuPathDBi | HostDB:ENSMUSG00000000738 |
Phylogenomic databases
eggNOGi | KOG0731, Eukaryota |
GeneTreei | ENSGT00940000156329 |
InParanoidi | Q3ULF4 |
OMAi | TRMKSMK |
OrthoDBi | 217929at2759 |
TreeFami | TF105003 |
Enzyme and pathway databases
BRENDAi | 3.4.24.B18, 3474 |
Reactomei | R-MMU-8949664, Processing of SMDT1 |
Miscellaneous databases
BioGRID-ORCSi | 234847, 0 hits in 75 CRISPR screens |
ChiTaRSi | Spg7, mouse |
PROi | PR:Q3ULF4 |
RNActi | Q3ULF4, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSMUSG00000000738, Expressed in retinal neural layer and 239 other tissues |
ExpressionAtlasi | Q3ULF4, baseline and differential |
Genevisiblei | Q3ULF4, MM |
Family and domain databases
Gene3Di | 1.20.58.760, 1 hit 3.40.50.300, 1 hit |
HAMAPi | MF_01458, FtsH, 1 hit |
InterProi | View protein in InterPro IPR003593, AAA+_ATPase IPR041569, AAA_lid_3 IPR003959, ATPase_AAA_core IPR005936, FtsH IPR027417, P-loop_NTPase IPR011546, Pept_M41_FtsH_extracell IPR000642, Peptidase_M41 IPR037219, Peptidase_M41-like |
Pfami | View protein in Pfam PF00004, AAA, 1 hit PF17862, AAA_lid_3, 1 hit PF06480, FtsH_ext, 1 hit PF01434, Peptidase_M41, 1 hit |
SMARTi | View protein in SMART SM00382, AAA, 1 hit |
SUPFAMi | SSF140990, SSF140990, 1 hit SSF52540, SSF52540, 1 hit |
TIGRFAMsi | TIGR01241, FtsH_fam, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | SPG7_MOUSE | |
Accessioni | Q3ULF4Primary (citable) accession number: Q3ULF4 Secondary accession number(s): B2RQY8 Q8R1K2 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | October 2, 2007 |
Last sequence update: | October 11, 2005 | |
Last modified: | May 25, 2022 | |
This is version 139 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program |
Miscellaneousi
Keywords - Technical termi
Direct protein sequencing, Reference proteomeDocuments
- Peptidase families
Classification of peptidase families and list of entries - MGD cross-references
Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot - SIMILARITY comments
Index of protein domains and families