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http://purl.uniprot.org/citations/11101512http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/11101512http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/11101512http://www.w3.org/2000/01/rdf-schema#comment"Tim8 and Tim13 are non-essential, conserved proteins of the mitochondrial intermembrane space, which are organized in a hetero-oligomeric complex. They are structurally related to Tim9 and Tim10, essential components of the import machinery for mitochondrial carrier proteins. Here we show that the TIM8-13 complex interacts with translocation intermediates of Tim23, which are partially translocated across the outer membrane but not with fully imported or assembled Tim23. The TIM8-13 complex binds to the N-terminal or intermediate domain of Tim23. It traps the incoming precursor in the intermembrane space thereby preventing retrograde translocation. The TIM8-13 complex is strictly required for import of Tim23 under conditions when a low membrane potential exists in the mitochondria. The human homologue of Tim8 is encoded by the DDP1 (deafness/dystonia peptide 1) gene, which is associated with the Mohr-Tranebjaerg syndrome (MTS), a progressive neurodegenerative disorder leading to deafness. It is demonstrated that import of human Tim23 is dependent on a high membrane potential. A mechanism to explain the pathology of MTS is discussed."xsd:string
http://purl.uniprot.org/citations/11101512http://purl.org/dc/terms/identifier"doi:10.1093/emboj/19.23.6392"xsd:string
http://purl.uniprot.org/citations/11101512http://purl.org/dc/terms/identifier"doi:10.1093/emboj/19.23.6392"xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/author"Neupert W."xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/author"Neupert W."xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/author"Bauer M.F."xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/author"Bauer M.F."xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/author"Brunner M."xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/author"Brunner M."xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/author"Paschen S.A."xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/author"Paschen S.A."xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/author"Kaldi K."xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/author"Kaldi K."xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/author"Rothbauer U."xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/author"Rothbauer U."xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/date"2000"xsd:gYear
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/date"2000"xsd:gYear
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/name"EMBO J."xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/name"EMBO J."xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/pages"6392-6400"xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/pages"6392-6400"xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/title"The role of the TIM8-13 complex in the import of Tim23 into mitochondria."xsd:string
http://purl.uniprot.org/citations/11101512http://purl.uniprot.org/core/title"The role of the TIM8-13 complex in the import of Tim23 into mitochondria."xsd:string