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Protein

Paraplegin

Gene

Spg7

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

ATP-dependent zinc metalloprotease.Curated

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: MGI
  • cell adhesion Source: MGI
  • mitochondrion organization Source: MGI
  • nervous system development Source: GO_Central
  • proteolysis Source: GO_Central
  • regulation of cell adhesion Source: MGI
Complete GO annotation...

Keywords - Molecular functioni

Hydrolase, Metalloprotease, Protease

Keywords - Ligandi

ATP-binding, Metal-binding, Nucleotide-binding, Zinc

Enzyme and pathway databases

BRENDAi3.4.24.B18. 3474.

Protein family/group databases

MEROPSiM41.006.

Names & Taxonomyi

Protein namesi
Recommended name:
Paraplegin1 Publication (EC:3.4.24.-)
Alternative name(s):
Spastic paraplegia 7 proteinImported
Gene namesi
Name:Spg7Imported
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 8

Organism-specific databases

MGIiMGI:2385906. Spg7.

Subcellular locationi

  • Mitochondrion membrane By similarity; Multi-pass membrane protein By similarity

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transmembranei145 – 165HelicalSequence analysisAdd BLAST21
Transmembranei249 – 269HelicalSequence analysisAdd BLAST21

GO - Cellular componenti

  • axon cytoplasm Source: GOC
  • m-AAA complex Source: MGI
  • mitochondrion Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion

Pathology & Biotechi

Disruption phenotypei

Mice are affected by a distal axonopathy of spinal and peripheral axons, characterized by axonal swelling and degeneration. Mitochondrial morphological abnormalities occurr in synaptic terminals and in distal regions of axons long before the first signs of swelling and degeneration and correlate with onset of motor impairment during a rotarod test.1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00003049321 – 781ParapleginAdd BLAST781

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei505Nitrated tyrosineCombined sources1

Keywords - PTMi

Nitration

Proteomic databases

EPDiQ3ULF4.
MaxQBiQ3ULF4.
PaxDbiQ3ULF4.
PRIDEiQ3ULF4.

PTM databases

iPTMnetiQ3ULF4.
PhosphoSitePlusiQ3ULF4.

Expressioni

Gene expression databases

BgeeiENSMUSG00000000738.
CleanExiMM_SPG7.
ExpressionAtlasiQ3ULF4. baseline and differential.
GenevisibleiQ3ULF4. MM.

Interactioni

Subunit structurei

Interacts with AFG3L2; the interaction is required for the efficient assembly of mitochondrial complex I. Interacts with MAIP1.By similarity

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000104496.

Structurei

3D structure databases

ProteinModelPortaliQ3ULF4.
SMRiQ3ULF4.
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

eggNOGiKOG0731. Eukaryota.
COG0465. LUCA.
GeneTreeiENSGT00860000133879.
HOVERGENiHBG050184.
InParanoidiQ3ULF4.
KOiK09552.
OrthoDBiEOG091G03B4.

Family and domain databases

Gene3Di3.40.50.300. 1 hit.
HAMAPiMF_01458. FtsH. 1 hit.
InterProiIPR003593. AAA+_ATPase.
IPR003959. ATPase_AAA_core.
IPR005936. FtsH.
IPR027417. P-loop_NTPase.
IPR011546. Pept_M41_FtsH_extracell.
IPR000642. Peptidase_M41.
[Graphical view]
PfamiPF00004. AAA. 1 hit.
PF06480. FtsH_ext. 1 hit.
PF01434. Peptidase_M41. 1 hit.
[Graphical view]
SMARTiSM00382. AAA. 1 hit.
[Graphical view]
SUPFAMiSSF52540. SSF52540. 1 hit.
TIGRFAMsiTIGR01241. FtsH_fam. 1 hit.

Sequencei

Sequence statusi: Complete.

Q3ULF4-1 [UniParc]FASTAAdd to basket

« Hide

        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
Length:781
Mass (Da):85,996
Last modified:October 11, 2005 - v1
Checksum:i35CCFB8F24B249D8
GO

Sequence cautioni

The sequence AAH96690 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti165I → T in AAO21098 (Ref. 2) Curated1
Sequence conflicti310D → G in AAO21098 (Ref. 2) Curated1
Sequence conflicti471L → F in AAN03852 (PubMed:14722615).Curated1

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.
BC024466 mRNA. Translation: AAH24466.1.
BC024986 mRNA. Translation: AAH24986.1.
BC051051 mRNA. Translation: AAH51051.1.
BC096690 mRNA. Translation: AAH96690.1. Different initiation.
CCDSiCCDS40508.1.
RefSeqiNP_694816.3. NM_153176.4.
UniGeneiMm.292075.

Genome annotation databases

EnsembliENSMUST00000149248; ENSMUSP00000119552; ENSMUSG00000000738.
GeneIDi234847.
KEGGimmu:234847.
UCSCiuc009nuc.1. mouse.

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.
BC024466 mRNA. Translation: AAH24466.1.
BC024986 mRNA. Translation: AAH24986.1.
BC051051 mRNA. Translation: AAH51051.1.
BC096690 mRNA. Translation: AAH96690.1. Different initiation.
CCDSiCCDS40508.1.
RefSeqiNP_694816.3. NM_153176.4.
UniGeneiMm.292075.

3D structure databases

ProteinModelPortaliQ3ULF4.
SMRiQ3ULF4.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000104496.

Protein family/group databases

MEROPSiM41.006.

PTM databases

iPTMnetiQ3ULF4.
PhosphoSitePlusiQ3ULF4.

Proteomic databases

EPDiQ3ULF4.
MaxQBiQ3ULF4.
PaxDbiQ3ULF4.
PRIDEiQ3ULF4.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000149248; ENSMUSP00000119552; ENSMUSG00000000738.
GeneIDi234847.
KEGGimmu:234847.
UCSCiuc009nuc.1. mouse.

Organism-specific databases

CTDi6687.
MGIiMGI:2385906. Spg7.

Phylogenomic databases

eggNOGiKOG0731. Eukaryota.
COG0465. LUCA.
GeneTreeiENSGT00860000133879.
HOVERGENiHBG050184.
InParanoidiQ3ULF4.
KOiK09552.
OrthoDBiEOG091G03B4.

Enzyme and pathway databases

BRENDAi3.4.24.B18. 3474.

Miscellaneous databases

ChiTaRSiSpg7. mouse.
PROiQ3ULF4.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000000738.
CleanExiMM_SPG7.
ExpressionAtlasiQ3ULF4. baseline and differential.
GenevisibleiQ3ULF4. MM.

Family and domain databases

Gene3Di3.40.50.300. 1 hit.
HAMAPiMF_01458. FtsH. 1 hit.
InterProiIPR003593. AAA+_ATPase.
IPR003959. ATPase_AAA_core.
IPR005936. FtsH.
IPR027417. P-loop_NTPase.
IPR011546. Pept_M41_FtsH_extracell.
IPR000642. Peptidase_M41.
[Graphical view]
PfamiPF00004. AAA. 1 hit.
PF06480. FtsH_ext. 1 hit.
PF01434. Peptidase_M41. 1 hit.
[Graphical view]
SMARTiSM00382. AAA. 1 hit.
[Graphical view]
SUPFAMiSSF52540. SSF52540. 1 hit.
TIGRFAMsiTIGR01241. FtsH_fam. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiSPG7_MOUSE
AccessioniPrimary (citable) accession number: Q3ULF4
Secondary accession number(s): Q4V9T9
, Q80X42, Q811Y5, Q8K414, Q8R1A1, Q8R1K2
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 2, 2007
Last sequence update: October 11, 2005
Last modified: November 30, 2016
This is version 106 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. Peptidase families
    Classification of peptidase families and list of entries
  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.