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SPG7 [mitochondrial inner membrane]
Stable Identifier
R-HSA-8953712
Type
Protein [EntityWithAccessionedSequence]
Species
Homo sapiens
Compartment
mitochondrial inner membrane
Synonyms
fullName evidence="2"Paraplegin ecNumber3.4.24.-/ecNumber, SPG7_HUMAN
Locations in the PathwayBrowser
Expand all
Metabolism of proteins (Homo sapiens)
Mitochondrial protein degradation (Homo sapiens)
AFG3L2:SPG7 binds SMDT1 (EMRE) (Homo sapiens)
AFG3L2:SPG7:SMDT1 [mitochondrial inner membrane] (Homo sapiens)
AFG3L2:Zn2+:SPG7:Zn2+ heterohexamer [mitochondrial inner membrane] (Homo sapiens)
SPG7:Zn2+ [mitochondrial inner membrane] (Homo sapiens)
SPG7 [mitochondrial inner membrane] (Homo sapiens)
AFG3L2:Zn2+:SPG7:Zn2+ heterohexamer [mitochondrial inner membrane] (Homo sapiens)
SPG7:Zn2+ [mitochondrial inner membrane] (Homo sapiens)
SPG7 [mitochondrial inner membrane] (Homo sapiens)
AFG3L2:SPG7 degrades SMDT1 (EMRE) (Homo sapiens)
AFG3L2:SPG7:SMDT1 [mitochondrial inner membrane] (Homo sapiens)
AFG3L2:Zn2+:SPG7:Zn2+ heterohexamer [mitochondrial inner membrane] (Homo sapiens)
SPG7:Zn2+ [mitochondrial inner membrane] (Homo sapiens)
SPG7 [mitochondrial inner membrane] (Homo sapiens)
AFG3L2:Zn2+:SPG7:Zn2+ heterohexamer [mitochondrial inner membrane] (Homo sapiens)
SPG7:Zn2+ [mitochondrial inner membrane] (Homo sapiens)
SPG7 [mitochondrial inner membrane] (Homo sapiens)
SPG7:Zn2+ [mitochondrial inner membrane] (Homo sapiens)
SPG7 [mitochondrial inner membrane] (Homo sapiens)
Transport of small molecules (Homo sapiens)
Mitochondrial calcium ion transport (Homo sapiens)
Processing of SMDT1 (Homo sapiens)
AFG3L2 (m-AAA protease) degrades SMDT1 that is not assembled in MCU (Homo sapiens)
C2orf47:AFG3L2:SPG7:PHB:PHB2 [mitochondrial inner membrane] (Homo sapiens)
SPG7 [mitochondrial inner membrane] (Homo sapiens)
C2orf47:AFG3L2 binds the transit peptide of SMDT1 (Homo sapiens)
C2orf47:AFG3L2:SPG7:PHB:PHB2 [mitochondrial inner membrane] (Homo sapiens)
SPG7 [mitochondrial inner membrane] (Homo sapiens)
proSMDT1:C2orf47:AFG3L2:SPG7:PHB:PHB2 [mitochondrial inner membrane] (Homo sapiens)
C2orf47:AFG3L2:SPG7:PHB:PHB2 [mitochondrial inner membrane] (Homo sapiens)
SPG7 [mitochondrial inner membrane] (Homo sapiens)
PMPCA:PMPCB cleaves the transit peptide of proSMDT1 (proEMRE) (Homo sapiens)
C2orf47:AFG3L2:SPG7:PHB:PHB2 [mitochondrial inner membrane] (Homo sapiens)
SPG7 [mitochondrial inner membrane] (Homo sapiens)
proSMDT1:C2orf47:AFG3L2:SPG7:PHB:PHB2 [mitochondrial inner membrane] (Homo sapiens)
C2orf47:AFG3L2:SPG7:PHB:PHB2 [mitochondrial inner membrane] (Homo sapiens)
SPG7 [mitochondrial inner membrane] (Homo sapiens)
External Reference Information
External Reference
UniProt:Q9UQ90 SPG7
Gene Names
SPG7, CAR, PGN, CMAR
Chain
transit peptide:1-43, propeptide:44-105, chain:106-795
Reference Genes
BioGPS Gene:6687 SPG7
COSMIC (genes):SPG7 SPG7
CTD Gene:6687 SPG7
dbSNP Gene:6687 SPG7
ENSEMBL:ENSG00000197912 SPG7
HGNC:11237 SPG7
KEGG:hsa:6687 SPG7
Monarch:6687 SPG7
NCBI Gene:6687 SPG7
OMIM:602783 SPG7
UCSC:Q9UQ90 SPG7
Reference Transcript
RefSeq:NM_199367.2 SPG7
RefSeq:XM_017023599.1 SPG7
RefSeq:NM_003119.3 SPG7
Other Identifiers
101930112
11715993_s_at
11729499_at
1557409_PM_at
1557409_at
16821972
16821977
16822001
202104_PM_s_at
202104_s_at
214494_PM_s_at
214494_s_at
230884_PM_s_at
230884_s_at
230885_PM_at
230885_at
235325_PM_at
235325_at
238179_PM_at
238179_at
31673_s_at
3674060
3674061
3674063
3674065
3674066
3674067
3674068
3674069
3674072
3674074
3674075
3674076
3674077
3674078
3674079
3674080
3674081
3674082
3674083
3674084
3674085
3674086
3674087
3674088
3674089
3674090
3674091
3674092
3674093
3674094
3674095
3674096
3674098
3674099
3674100
3674101
3674102
3674103
3674104
3674105
3674106
3674107
3674108
3674109
3674110
3674111
3674112
3674113
3674114
3674115
3674116
3674117
3674118
3674119
3674120
3674121
3674122
3674123
3674124
3674125
3674126
3674127
3674128
3674129
3674130
3674131
3674133
3674134
3674135
3674139
3674140
3674142
3674143
3674144
3674145
3674147
3674148
3674149
3674150
3674151
3758833
45086_at
6687
74333_at
7997909
7997933
80422_at
84838_at
85222_r_at
ADDON000330.hg
ADDON002200.hg
A_14_P112688
A_14_P116582
A_14_P121120
A_21_P0008998
A_21_P0009117
A_23_P3627
A_23_P433990
A_24_P205120
A_24_P5305
A_32_P115446
A_32_P115451
A_33_P3381361
GE60332
GE627776
GE735082
GE84260
GE86385
GE86834
GO:0003824
GO:0004176
GO:0004222
GO:0005515
GO:0005524
GO:0005739
GO:0005743
GO:0005745
GO:0005757
GO:0006508
GO:0007005
GO:0007018
GO:0007399
GO:0008089
GO:0008233
GO:0008237
GO:0008270
GO:0016020
GO:0016787
GO:0016887
GO:0034982
GO:0043226
GO:0046872
GO:0046902
GO:0048856
GO:0051082
GO:0051604
GO:0061024
GO:0110097
GO:0140096
GO:0140657
GO:1902686
GO:1904115
HMNXSV003007620
HMNXSV003008712
HMNXSV003020529
HMNXSV003026490
HMNXSV003039907
HMNXSV003041374
HMNXSV003043515
Hs.148468.0.A1_3p_at
Hs.180842.3.A1_3p_s_at
Hs.183957.0.A1_3p_at
Hs.272480.0.S1_3p_s_at
Hs.296847.2.A1_3p_a_at
Hs2.406971.1.S1_3p_at
ILMN_1675583
ILMN_1697096
ILMN_1709039
ILMN_1754529
PH_hs_0023727
PH_hs_0037761
TC16000698.hg
TC16001712.hg
X65784_s_at
p20116
p20117
Participates
as a component of
SPG7:Zn2+ [mitochondrial inner membrane] (Homo sapiens)
C2orf47:AFG3L2:SPG7:PHB:PHB2 [mitochondrial inner membrane] (Homo sapiens)
Inferred To
ppgn-1 [mitochondrial inner membrane]
(Caenorhabditis elegans)
SPG7 [mitochondrial inner membrane]
(Bos taurus)
SPG7 [mitochondrial inner membrane]
(Canis familiaris)
SPG7 [mitochondrial inner membrane]
(Dictyostelium discoideum)
SPG7 [mitochondrial inner membrane]
(Gallus gallus)
SPG7 [mitochondrial inner membrane]
(Mus musculus)
SPG7 [mitochondrial inner membrane]
(Plasmodium falciparum)
SPG7 [mitochondrial inner membrane]
(Rattus norvegicus)
SPG7 [mitochondrial inner membrane]
(Saccharomyces cerevisiae)
SPG7 [mitochondrial inner membrane]
(Schizosaccharomyces pombe)
SPG7 [mitochondrial inner membrane]
(Sus scrofa)
Spg7 [mitochondrial inner membrane]
(Drosophila melanogaster)
rcaA [mitochondrial inner membrane]
(Dictyostelium discoideum)
spg7 [mitochondrial inner membrane]
(Xenopus tropicalis)
Homologues of SPG7 [mitochondrial inner membrane]
(Dictyostelium discoideum)
Homologues of SPG7 [mitochondrial inner membrane]
(Plasmodium falciparum)
Homologues of SPG7 [mitochondrial inner membrane]
(Saccharomyces cerevisiae)
Cross References
ENSEMBL
ENSP00000495795
,
ENSP00000494119
,
ENST00000645818
,
ENST00000341316
,
ENST00000646263
,
ENSP00000341157
OpenTargets
ENSG00000197912
HPA
ENSG00000197912-SPG7
PRO
Q9UQ90
Pharos - Targets
Q9UQ90
GlyGen
Q9UQ90
Orphanet
SPG7
PDB
2QZ4
Interactors (7)
Accession
#Entities
Entities
Confidence Score
Evidence (IntAct)
UniProt:P61289 PSME3
1
PSME3 [cytosol]
(R-HSA-68816)
0.556
3
UniProt:Q7Z3S9 NOTCH2NLA
3
NOTCH2NLA [extracellular region]
(R-HSA-9908935)
NOTCH2NLA(1-?) [extracellular region]
(R-HSA-9911493)
NOTCH2NLA(?-236) [extracellular region]
(R-HSA-9911490)
0.556
3
UniProt:Q5VWX1 KHDRBS2
2
KHDRBS2 [nucleoplasm]
(R-HSA-8849028)
p-Y-KHDRBS2 [nucleoplasm]
(R-HSA-8849031)
0.556
3
UniProt:P0DPK4 NOTCH2NLC
1
NOTCH2NLC [extracellular region]
(R-HSA-9908932)
0.556
3
UniProt:P61978 HNRNPK
2
HNRNPK [nucleoplasm]
(R-HSA-71945)
polySUMO2-K422-HNRNPK [nucleoplasm]
(R-HSA-4570532)
0.556
6
UniProt:Q99677 LPAR4
1
LPAR4 [plasma membrane]
(R-HSA-418020)
0.527
2
UniProt:P35414 APLNR
1
APLNR [plasma membrane]
(R-HSA-374259)
0.527
2
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