J PROTEIN MUTATIONS AND RESULTING PROTEOSTASIS COLLAPSE
Despite a century of intensive investigation the effective treatment of protein aggregation diseases remains elusive. Ordinarily, molecular chaperones ensure that proteins maintain their functional conformation. The appearance of misfolded proteins that aggregate implies the collapse of the cellula...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
Frontiers Media S.A.
2014-07-01
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| Series: | Frontiers in Cellular Neuroscience |
| Subjects: | |
| Online Access: | http://journal.frontiersin.org/Journal/10.3389/fncel.2014.00191/full |
| _version_ | 1818423032221270016 |
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| author | Carolina eKoutras Janice Eva Arlee Braun |
| author_facet | Carolina eKoutras Janice Eva Arlee Braun |
| author_sort | Carolina eKoutras |
| collection | DOAJ |
| description | Despite a century of intensive investigation the effective treatment of protein aggregation diseases remains elusive. Ordinarily, molecular chaperones ensure that proteins maintain their functional conformation. The appearance of misfolded proteins that aggregate implies the collapse of the cellular chaperone quality control network. That said, the cellular chaperone network is extensive and functional information regarding the detailed action of specific chaperones is not yet available. J proteins (DnaJ/Hsp40) are a family of chaperone cofactors that harness Hsc70 (heat shock cognate protein of 70kDa) for diverse conformational cellular tasks and, as such, represent novel clinically relevant targets for diseases resulting from the disruption of proteostasis. Here we review incisive reports identifying mutations in individual J protein chaperones and the proteostasis collapse that ensues. |
| first_indexed | 2024-12-14T13:35:42Z |
| format | Article |
| id | doaj.art-5be0a3cf96fb4c87a7c0b2b6b58d5f00 |
| institution | Directory Open Access Journal |
| issn | 1662-5102 |
| language | English |
| last_indexed | 2024-12-14T13:35:42Z |
| publishDate | 2014-07-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Cellular Neuroscience |
| spelling | doaj.art-5be0a3cf96fb4c87a7c0b2b6b58d5f002022-12-21T22:59:35ZengFrontiers Media S.A.Frontiers in Cellular Neuroscience1662-51022014-07-01810.3389/fncel.2014.0019197458J PROTEIN MUTATIONS AND RESULTING PROTEOSTASIS COLLAPSECarolina eKoutras0Janice Eva Arlee Braun1University of CalgaryUniversity of CalgaryDespite a century of intensive investigation the effective treatment of protein aggregation diseases remains elusive. Ordinarily, molecular chaperones ensure that proteins maintain their functional conformation. The appearance of misfolded proteins that aggregate implies the collapse of the cellular chaperone quality control network. That said, the cellular chaperone network is extensive and functional information regarding the detailed action of specific chaperones is not yet available. J proteins (DnaJ/Hsp40) are a family of chaperone cofactors that harness Hsc70 (heat shock cognate protein of 70kDa) for diverse conformational cellular tasks and, as such, represent novel clinically relevant targets for diseases resulting from the disruption of proteostasis. Here we review incisive reports identifying mutations in individual J protein chaperones and the proteostasis collapse that ensues.http://journal.frontiersin.org/Journal/10.3389/fncel.2014.00191/fullHSP40 Heat-Shock Proteinsneurodegeneration and neuroprotectioncysteine string proteinDnaJC5J ProteinDnaJC29 |
| spellingShingle | Carolina eKoutras Janice Eva Arlee Braun J PROTEIN MUTATIONS AND RESULTING PROTEOSTASIS COLLAPSE Frontiers in Cellular Neuroscience HSP40 Heat-Shock Proteins neurodegeneration and neuroprotection cysteine string protein DnaJC5 J Protein DnaJC29 |
| title | J PROTEIN MUTATIONS AND RESULTING PROTEOSTASIS COLLAPSE |
| title_full | J PROTEIN MUTATIONS AND RESULTING PROTEOSTASIS COLLAPSE |
| title_fullStr | J PROTEIN MUTATIONS AND RESULTING PROTEOSTASIS COLLAPSE |
| title_full_unstemmed | J PROTEIN MUTATIONS AND RESULTING PROTEOSTASIS COLLAPSE |
| title_short | J PROTEIN MUTATIONS AND RESULTING PROTEOSTASIS COLLAPSE |
| title_sort | j protein mutations and resulting proteostasis collapse |
| topic | HSP40 Heat-Shock Proteins neurodegeneration and neuroprotection cysteine string protein DnaJC5 J Protein DnaJC29 |
| url | http://journal.frontiersin.org/Journal/10.3389/fncel.2014.00191/full |
| work_keys_str_mv | AT carolinaekoutras jproteinmutationsandresultingproteostasiscollapse AT janiceevaarleebraun jproteinmutationsandresultingproteostasiscollapse |