Conserved Protein Domain Family
OPA3

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pfam07047: OPA3 (This model is not part of the current CDD release)
Optic atrophy 3 protein (OPA3)
This family consists of several optic atrophy 3 (OPA3) proteins. OPA3 deficiency causes type III 3-methylglutaconic aciduria (MGA) in humans. This disease manifests with early bilateral optic atrophy, spasticity, extrapyramidal dysfunction, ataxia, and cognitive deficits, but normal longevity.
Statistics
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PSSM-Id: 517533
Aligned: 129 rows
Threshold Bit Score: 71.3901
Created: 14-Feb-2025
Updated: 28-Apr-2025
Structure
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Aligned Rows:
PubMed ReferencesClick to see Conserved Features Help

Sequence Alignment
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Format: Row Display: Color Bits: Type Selection:
XP_014172718    7 FKLAALFVRHISKYGANQIKIQAHE-------HQRFR-RQAARYGQAIHQLNMRL----NVAVLRNyd-aeKRarekaea 73 
13_pfamImport   1 AKLLPLVFKIVTNPVVKVLQRNVSK-------SQFWEhRVFLPMARSYNKASVRV----RLWSQGIhr-nkEQv------ 62 
XP_004225843    8 AKLLPEIFKTVTKPMVNALKRNVRK-------SPFWKdSVFIPMAQSYNKASVRV----RLWSQGIhr-nkEQv------ 69 
XP_001023042    5 LKALSLVGKVFSRPIVVIFKVGIQK-------IGGTPrETLVRWGNIIYNWEAWI----NRRFLGYeg-kvKE------- 65 
A0CU80          5 KKLAALFIRTFSKPVANIIKRYALNnnnnrsfGRRIVrNSFIFLGNKYHAFDTYL----ERASIGQtn---qQ------- 70  Paramecium tet...
XP_001016702    5 FKLFSLFVRILARPVIARTKAHTSSkl----qNQKFR-VFFVWLGNKAHRINVYI----NRTFLKLnn-qdFK------- 67 
A0CNE2          5 LKLISLIVRVFARPIITSRKEVSIEkvl--msKQRFLtQQIQNLGNYYYYTDQKI----DRMYLNQnl-hdEY------- 70  Paramecium tet...
A0BUL1          5 FKVFSLIVRVFARPVIARTKAAHLKkaq--sgHTTWIkQFYVRLGNFQHKWDQKI----DSKFMGIdkkssDF------- 71  Paramecium tet...
XP_032510522    9 FRLATLLARQLSSPVATRIKRYAVG-------NPKVGgRVCRCVAQSYHECEWMA----RTWALKMag-isLPk------ 70 
XP_008206277    9 SALLTQLFRQLTKPASQRIVDYAAR-------HPSFRrYLLLAPGRLYHRLESHLerlkAAPMVASka-ekKP------- 73 
XP_014172718   74 aeaptvktkeqaereerlkqkeaadrakygttakeahpatvimrtsdasipaasaaaatatsstsssadstrgpthftsv 153
13_pfamImport     --------------------------------------------------------------------------------    
XP_004225843      --------------------------------------------------------------------------------    
XP_001023042      --------------------------------------------------------------------------------    
A0CU80            --------------------------------------------------------------------------------     Paramecium tet...
XP_001016702      --------------------------------------------------------------------------------    
A0CNE2            --------------------------------------------------------------------------------     Paramecium tet...
A0BUL1            --------------------------------------------------------------------------------     Paramecium tet...
XP_032510522      --------------------------------------------------------------------------------    
XP_008206277      --------------------------------------------------------------------------------    
XP_014172718  154 wkRKFRALPEAKAVDL---------FADVVGAAFILVIASALVLYEYYRSASKP--DanlANLERIKDLngqLTELRAAE 222
13_pfamImport  63 --EKSARLSDEAALEL---------GAEIVANSATFIVGLLAIIMQQSIVAATE--K---KKEKEEQLE---TKRIEENL 123
XP_004225843   70 --ERSAHMNDEAALEL---------GAEIVANSATFIVGLLAIIMQQSIAAATE--K---KKEKQEEQE---LMKMETNI 130
XP_001023042   66 -----VLLSDEKALEN---------FANFFIEVVIVYGVIGYITISQGIQKHRE------MQEEKERK-----DRLEEEV 120
A0CU80         71 --FFIKPLTDEAAFMK---------GTDLFSDLFIYACVLGLPLYEIMRQSNES--A---KKEAIQDEK---LHKLSKEV 131 Paramecium tet...
XP_001016702   68 ----VQDLSEPAALEL---------GVETFYEVIIYACILGLPIIEIYRAQVES--N---EKSEKQKQV---MEGIHTRL 126
A0CNE2         71 -----DKLNDEDALKLvilitiilqGTEFLLEMIIYTILILVSLFEIYKTYFEQqeR---------------LQNMNNTI 130 Paramecium tet...
A0BUL1         72 ---FFKPLNDELALEK---------GVEFFYEILIYALLITLPTYEMYSAQQDS--K---KKSEQNTQK---LNNLMKQI 131 Paramecium tet...
XP_032510522   71 -nARAPPLRDQTALNL---------GGDLLGEIIIFTIGSLIIIFEVNRQADIK--E---TKELDEKSE---WLAIMLSL 132
XP_008206277   74 ---KVKALKDEEAAML---------GSQLMIEIVIFALLALILTCDWLRSQRKS--A---LEEDRRRAE---FAELERQK 133
XP_014172718  223 DEH 225
13_pfamImport 124 IDL 126
XP_004225843  131 LEL 133
XP_001023042  121 YYL 123
A0CU80        132 EEL 134 Paramecium tetraurelia
XP_001016702  127 QNL 129
A0CNE2        131 KNL 133 Paramecium tetraurelia
A0BUL1        132 EEN 134 Paramecium tetraurelia
XP_032510522  133 EEI 135
XP_008206277  134 SEA 136
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