1ON4,1WP0,1XZO


Conserved Protein Domain Family
SCO
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cd02968: SCO 
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SCO (an acronym for Synthesis of Cytochrome c Oxidase) family; composed of proteins similar to Sco1, a membrane-anchored protein possessing a soluble domain with a TRX fold. Members of this family are required for the proper assembly of cytochrome c oxidase (COX). They contain a metal binding motif, typically CXXXC, which is located in a flexible loop. COX, the terminal enzyme in the respiratory chain, is imbedded in the inner mitochondrial membrane of all eukaryotes and in the plasma membrane of some prokaryotes. It is composed of two subunits, COX I and COX II. It has been proposed that Sco1 specifically delivers copper to the CuA site, a dinuclear copper center, of the COX II subunit. Mutations in human Sco1 and Sco2 cause fatal infantile hepatoencephalomyopathy and cardioencephalomyopathy, respectively. Both disorders are associated with severe COX deficiency in affected tissues. More recently, it has been argued that the redox sensitivity of the copper binding properties of Sco1 implies that it participates in signaling events rather than functioning as a chaperone that transfers copper to COX II.
Links
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Statistics
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PSSM-Id: 239266
Aligned: 159 rows
Threshold Bit Score: 76.1026
Created: 8-Dec-2004
Updated: 2-Oct-2020
Structure
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Program:
Drawing:
Aligned Rows:
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Cu(I) binding
Conserved site includes 3 residues -Click on image for an interactive view with Cn3D
Feature 1:Cu(I) binding site [ion binding site]
Evidence:
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Sequence Alignment
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Format: Row Display: Color Bits: Type Selection: 
Feature 1                                         #   #                                         
1ON4_A       12 EVEPFTFQNQDgKNVSLEsl-kGEVWLADFIFTNCEtiCPPMTAHMTDLQKKLKaen---idvRIISFSVDPenDKPKQL 87  Bacillus subtilis
T18205       33 SVENAALESVDgSTFQLHd---QKIKLVVFFYTHCPdiCPMTLFDLEQVKKELEtknlfkekvRFISITLDPevDTQERL 109 Bacillus sp.
AAC07905     48 FVPNVEVILEDgTKTDLYtlikEKPTILLLSYYTCEgtCPVRVDNLKKLIDETDlkd---rdfMVLNLSFDEr-DDLQTL 123 Aquifex aeolicus...
AAC07906     35 VPGDITLVDSYgNEFQLKnl-kGKPIILSPIYTHCRaaCPLITKSLLKVIPKLGtpg---kdfWVITFTFDPk-DTLEDI 109 Aquifex aeolicus...
NP_969406    47 YNLNSSLLDEDgKVFQLEky-rGKPVVISMAYTSCVytCPLILAQMQQLEKALAeqgk--kdvRFVLVSFDPakDTPKVL 123 Bdellovibrio bac...
YP_105476    46 YQANATLTDQDgKTFRLAdl-rGTPTLVSMFYTSCKmvCPILFETIGLTLDAGGepar--kriKVVAVTVDPdrDSVAVL 122 Burkholderia mal...
AAQ59681     32 YRLEAPLTTQRgDALTFSdl-aGKPALITMFYGDCKlaCPIVMEGVKSIIAALParer--yrpTVLMVSLNPgtDTPASL 108 Chromobacterium ...
ZP_00049083   1 MVGSHQLLDQDgQPVDITry-rGKPVVISMIYTACDhtCPVTTQNVARAVAGARkalg-kdsfQVLSIGFDTvrDTPEAL 78  Magnetospirillum...
YP_146528    33 LPNDVTMETAWgKSYEFGdl-pPKVRLVEFIYTNCPdvCPNTTAQMAKLRDRLKqagvfgskvEFITITIDParDTNEKL 111 Geobacillus kaus...
YP_155154    56 YQIDATWNTHKnSQLKLSel-eGHPVILSMIYGSCTtaCPVLVHDVKRLYSKLDedtk--qqvKLVMVSFDTdrDTPEAL 132 Idiomarina loihi...

Feature 1                                                        #                   
1ON4_A       88 KKFAAnypl--sfdnWDFLTGysqseieEFALKSFKAIVkkpegedqviHQSSFYLVGpdGKVLKDYNG 154 Bacillus subtilis
T18205      110 QEYAQnfsv--dhagWMMLRGdts--ttEQIAKDFKMIYqknes-gfvtHSTTMYLVDanNKIRAYHDM 173 Bacillus sp.
AAC07905    124 KEFVKlhgp--ftkhWVFGIIdke--niEKLTNSVGFKFffverdktfvHPNVYIFLSpeGRITRYLWG 188 Aquifex aeolicus VF5
AAC07906    110 KRFQKeygi--dgkgWKVVKAkts-edlFKLLDAIDFRFmtag--ndfiHPNVVVVLSpeLQIKDYIYG 173 Aquifex aeolicus VF5
NP_969406   124 KDYAKkkkl---stqWNLLTSksd-kepREIASVLGIKYskveg-gdydHSFIITVLDaeGVPRGRQVG 187 Bdellovibrio bacteriovorus ...
YP_105476   123 RKTANahgl---darWRLVRTdrd--stRTVAALLGIQYrklas-gefnHSSTIVLLDadGRIVAHTNT 185 Burkholderia mallei ATCC 23344
AAQ59681    109 SHLAGehgi--rarnWLLAVGksd-edtRGVAAALGIRYrrlds-geinHSTRIVVADkdGRISASTED 173 Chromobacterium violaceum A...
ZP_00049083  79 KVYAKqqgi--dtggWSFLAGnpa--tmEALAGDLGFSWyvts--rgfdHIAQTTVLDgqGRIYRQVYG 141 Magnetospirillum magnetotac...
YP_146528   112 QTYANafgvmsdgqgWVFLRGsea--dtKKVADAFHFQYrdpgn-gmivHTTLAYLLGrnGRVVKQIGM 177 Geobacillus kaustophilus HT...
YP_155154   133 ADYVKqyql--nndnWLFLNGseq--diRTLATVLGVRYrqrpd-gdfdHSNLITILDkqGLIVERIEG 196 Idiomarina loihiensis L2TR

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