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Protein

Paraplegin

Gene

Spg7

Organism
Rattus norvegicus (Rat)
Status
Reviewed-Annotation score: -Experimental evidence at transcript leveli

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 (PubMed:26387735).Curated1 Publication

Cofactori

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

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei312ATP; via amide nitrogen and carbonyl oxygenBy similarity1
Binding sitei492ATPBy similarity1
Metal bindingi574Zinc; catalyticBy similarity1
Active sitei575By similarity1
Metal bindingi578Zinc; catalyticBy similarity1
Metal bindingi650Zinc; catalyticBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi349 – 357ATPBy similarity9

GO - Molecular functioni

GO - Biological processi

  • anterograde axonal transport Source: Ensembl
  • mitochondrial outer membrane permeabilization involved in programmed cell death Source: UniProtKB
  • regulation of mitochondrial membrane permeability Source: UniProtKB

Keywordsi

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

Enzyme and pathway databases

ReactomeiR-RNO-8949664 Processing of SMDT1

Protein family/group databases

MEROPSiM41.006

Names & Taxonomyi

Protein namesi
Recommended name:
Paraplegin (EC:3.4.24.-)
Gene namesi
Name:Spg7
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Chromosome 19

Organism-specific databases

RGDi727940 Spg7

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 domaini106 – 144Mitochondrial matrixCuratedAdd BLAST39
Transmembranei145 – 165HelicalSequence analysisAdd BLAST21
Topological domaini166 – 248Mitochondrial intermembraneCuratedAdd BLAST83
Transmembranei249 – 269HelicalSequence analysisAdd BLAST21
Topological domaini270 – 781Mitochondrial matrixCuratedAdd BLAST512

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 43MitochondrionBy similarityAdd BLAST43
PropeptideiPRO_000044230844 – 105Removed in mature formBy similarityAdd BLAST62
ChainiPRO_0000304933106 – 781ParapleginAdd BLAST676

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei505Nitrated tyrosineBy similarity1

Post-translational modificationi

Upon import into the mitochondrion, the N-terminal transit peptide is cleaved by the mitochondrial-processing peptidase (MPP) to generate an intermediate form which undergoes a second proteolytic cleavage mediated by proteases AFG3L1 and/or AFG3L2 removing an additional N-terminal fragment to generate the proteolytically active mature form.By similarity

Keywords - PTMi

Nitration

Proteomic databases

PaxDbiQ7TT47
PRIDEiQ7TT47

PTM databases

SwissPalmiQ7TT47

Expressioni

Gene expression databases

BgeeiENSRNOG00000015150
ExpressionAtlasiQ7TT47 baseline and differential
GenevisibleiQ7TT47 RN

Interactioni

Subunit structurei

Forms heterooligomers with AFG3L1 and AFG3L2. Component of the mitochondrial permeability transition pore complex (mPTPC), at least composed of SPG7, VDAC1 and PPIF. Interacts with AFG3L2; the interaction is required for the efficient assembly of mitochondrial complex I. Interacts with AFG3L1. Interacts with MAIP1. Interacts with VDAC1 and PPIF.By similarity

Protein-protein interaction databases

STRINGi10116.ENSRNOP00000048848

Structurei

3D structure databases

ProteinModelPortaliQ7TT47
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni701 – 781Interaction with PPIFBy similarityAdd BLAST81

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

Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0731 Eukaryota
COG0465 LUCA
GeneTreeiENSGT00890000139410
HOGENOMiHOG000217277
HOVERGENiHBG050184
InParanoidiQ7TT47
KOiK09552
OMAiKMEVKEF

Family and domain databases

HAMAPiMF_01458 FtsH, 1 hit
InterProiView protein in InterPro
IPR003593 AAA+_ATPase
IPR003959 ATPase_AAA_core
IPR005936 FtsH
IPR027417 P-loop_NTPase
IPR011546 Pept_M41_FtsH_extracell
IPR000642 Peptidase_M41
IPR037219 Peptidase_M41-like
PfamiView protein in Pfam
PF00004 AAA, 1 hit
PF06480 FtsH_ext, 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

Sequences (2)i

Sequence statusi: Complete.

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

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

Isoform 1 (identifier: Q7TT47-1) [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
MAAALLLLRA LRQSPEPGPW RLWAQLSGRS PGLFSGAGGR RPYVVRGTPI
60 70 80 90 100
GLAAAGGHTP QSLLLRILTP SFEGVSGLLL KRHIVPSAIR LWQLSGSTLY
110 120 130 140 150
FNTSGLKQKN KDDDKPKGKA PEDDEEERRR KEREDQMYRE RLRTLFIIAI
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 DGMGTADHVI
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 EGLVGIGRRP FSQGLQQMMD
710 720 730 740 750
HEARLLVARA YRHTEKVLLD NLDKLQALAN ALLEKEVINY EDIEALIGPP
760 770 780
PHGPKKMIAP QKWIDAEKEK QASGEEEAPA P
Length:781
Mass (Da):86,103
Last modified:November 22, 2017 - v2
Checksum:iF4C5D4673F5BCFD2
GO
Isoform 2 (identifier: Q7TT47-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     517-554: Missing.

Show »
Length:743
Mass (Da):82,036
Checksum:iF7B157FF357BE6A3
GO

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_059223517 – 554Missing in isoform 2. 1 PublicationAdd BLAST38

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY278739 mRNA Translation: AAP35059.1
AC119635 Genomic DNA No translation available.
AC141337 Genomic DNA No translation available.
RefSeqiNP_852053.1, NM_181388.2
XP_008770841.1, XM_008772619.2 [Q7TT47-1]
UniGeneiRn.16437

Genome annotation databases

EnsembliENSRNOT00000091712; ENSRNOP00000073276; ENSRNOG00000015150 [Q7TT47-1]
GeneIDi353231
KEGGirno:353231
UCSCiRGD:727940 rat [Q7TT47-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiSPG7_RAT
AccessioniPrimary (citable) accession number: Q7TT47
Secondary accession number(s): A0A0G2K536
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 2, 2007
Last sequence update: November 22, 2017
Last modified: May 23, 2018
This is version 116 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

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