SPG7 Variant Escapes Phosphorylation-Regulated Processing by AFG3L2, Elevates Mitochondrial ROS, and Is Associated with Multiple Clinical Phenotypes

Mitochondrial production of reactive oxygen species (ROS) affects many processes in health and disease. SPG7 assembles with AFG3L2 into the mAAA protease at the inner membrane of mitochondria, degrades damaged proteins, and regulates the synthesis of mitochondrial ribosomes. SPG7 is cleaved and acti...
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Autor*in:	Naif A.M. Almontashiri [verfasserIn] Hsiao-Huei Chen [verfasserIn] Ryan J. Mailloux [verfasserIn] Takashi Tatsuta [verfasserIn] Allen C.T. Teng [verfasserIn] Ahmad B. Mahmoud [verfasserIn] Tiffany Ho [verfasserIn] Nicolas A.S. Stewart [verfasserIn] Peter Rippstein [verfasserIn] Mary Ellen Harper [verfasserIn] Robert Roberts [verfasserIn] Christina Willenborg [verfasserIn] Jeanette Erdmann [verfasserIn] Annalisa Pastore [verfasserIn] Heidi M. McBride [verfasserIn] Thomas Langer [verfasserIn] Alexandre F.R. Stewart [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2014

Übergeordnetes Werk:	In: Cell Reports - Elsevier, 2015, 7(2014), 3, Seite 834-847
Übergeordnetes Werk:	volume:7 ; year:2014 ; number:3 ; pages:834-847

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc

DOI / URN:	10.1016/j.celrep.2014.03.051

Katalog-ID:	DOAJ001489925

Internformat


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520			\|a Mitochondrial production of reactive oxygen species (ROS) affects many processes in health and disease. SPG7 assembles with AFG3L2 into the mAAA protease at the inner membrane of mitochondria, degrades damaged proteins, and regulates the synthesis of mitochondrial ribosomes. SPG7 is cleaved and activated by AFG3L2 upon assembly. A variant in SPG7 that replaces arginine 688 with glutamine (Q688) is associated with several phenotypes, including toxicity of chemotherapeutic agents, type 2 diabetes mellitus, and (as reported here) coronary artery disease. We demonstrate that SPG7 processing is regulated by tyrosine phosphorylation of AFG3L2. Carriers of Q688 bypass this regulation and constitutively process and activate SPG7 mAAA protease. Cells expressing Q688 produce higher ATP levels and ROS, promoting cell proliferation. Our results thus reveal an unexpected link between the phosphorylation-dependent regulation of the mitochondria mAAA protease affecting ROS production and several clinical phenotypes.
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700	0		\|a Allen C.T. Teng \|e verfasserin \|4 aut
700	0		\|a Ahmad B. Mahmoud \|e verfasserin \|4 aut
700	0		\|a Tiffany Ho \|e verfasserin \|4 aut
700	0		\|a Nicolas A.S. Stewart \|e verfasserin \|4 aut
700	0		\|a Peter Rippstein \|e verfasserin \|4 aut
700	0		\|a Mary Ellen Harper \|e verfasserin \|4 aut
700	0		\|a Robert Roberts \|e verfasserin \|4 aut
700	0		\|a Christina Willenborg \|e verfasserin \|4 aut
700	0		\|a Jeanette Erdmann \|e verfasserin \|4 aut
700	0		\|a Annalisa Pastore \|e verfasserin \|4 aut
700	0		\|a Heidi M. McBride \|e verfasserin \|4 aut
700	0		\|a Thomas Langer \|e verfasserin \|4 aut
700	0		\|a Alexandre F.R. Stewart \|e verfasserin \|4 aut
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Indexfelder

author_variant	n a na h h c hhc r m rm t t tt a t at a m am t h th n s ns p r pr m h mh r r rr c w cw j e je a p ap h m hm t l tl a s as
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publishDate	2014
allfields	10.1016/j.celrep.2014.03.051 doi (DE-627)DOAJ001489925 (DE-599)DOAJaebe476dd1c04886a50974f3c1065d37 DE-627 ger DE-627 rakwb eng QH301-705.5 Naif A.M. Almontashiri verfasserin aut SPG7 Variant Escapes Phosphorylation-Regulated Processing by AFG3L2, Elevates Mitochondrial ROS, and Is Associated with Multiple Clinical Phenotypes 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Mitochondrial production of reactive oxygen species (ROS) affects many processes in health and disease. SPG7 assembles with AFG3L2 into the mAAA protease at the inner membrane of mitochondria, degrades damaged proteins, and regulates the synthesis of mitochondrial ribosomes. SPG7 is cleaved and activated by AFG3L2 upon assembly. A variant in SPG7 that replaces arginine 688 with glutamine (Q688) is associated with several phenotypes, including toxicity of chemotherapeutic agents, type 2 diabetes mellitus, and (as reported here) coronary artery disease. We demonstrate that SPG7 processing is regulated by tyrosine phosphorylation of AFG3L2. Carriers of Q688 bypass this regulation and constitutively process and activate SPG7 mAAA protease. Cells expressing Q688 produce higher ATP levels and ROS, promoting cell proliferation. Our results thus reveal an unexpected link between the phosphorylation-dependent regulation of the mitochondria mAAA protease affecting ROS production and several clinical phenotypes. Biology (General) Hsiao-Huei Chen verfasserin aut Ryan J. Mailloux verfasserin aut Takashi Tatsuta verfasserin aut Allen C.T. Teng verfasserin aut Ahmad B. Mahmoud verfasserin aut Tiffany Ho verfasserin aut Nicolas A.S. Stewart verfasserin aut Peter Rippstein verfasserin aut Mary Ellen Harper verfasserin aut Robert Roberts verfasserin aut Christina Willenborg verfasserin aut Jeanette Erdmann verfasserin aut Annalisa Pastore verfasserin aut Heidi M. McBride verfasserin aut Thomas Langer verfasserin aut Alexandre F.R. Stewart verfasserin aut In Cell Reports Elsevier, 2015 7(2014), 3, Seite 834-847 (DE-627)684964562 (DE-600)2649101-1 22111247 nnns volume:7 year:2014 number:3 pages:834-847 https://doi.org/10.1016/j.celrep.2014.03.051 kostenfrei https://doaj.org/article/aebe476dd1c04886a50974f3c1065d37 kostenfrei http://www.sciencedirect.com/science/article/pii/S2211124714002484 kostenfrei https://doaj.org/toc/2211-1247 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2110 GBV_ILN_2112 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4393 GBV_ILN_4700 AR 7 2014 3 834-847
spelling	10.1016/j.celrep.2014.03.051 doi (DE-627)DOAJ001489925 (DE-599)DOAJaebe476dd1c04886a50974f3c1065d37 DE-627 ger DE-627 rakwb eng QH301-705.5 Naif A.M. Almontashiri verfasserin aut SPG7 Variant Escapes Phosphorylation-Regulated Processing by AFG3L2, Elevates Mitochondrial ROS, and Is Associated with Multiple Clinical Phenotypes 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Mitochondrial production of reactive oxygen species (ROS) affects many processes in health and disease. SPG7 assembles with AFG3L2 into the mAAA protease at the inner membrane of mitochondria, degrades damaged proteins, and regulates the synthesis of mitochondrial ribosomes. SPG7 is cleaved and activated by AFG3L2 upon assembly. A variant in SPG7 that replaces arginine 688 with glutamine (Q688) is associated with several phenotypes, including toxicity of chemotherapeutic agents, type 2 diabetes mellitus, and (as reported here) coronary artery disease. We demonstrate that SPG7 processing is regulated by tyrosine phosphorylation of AFG3L2. Carriers of Q688 bypass this regulation and constitutively process and activate SPG7 mAAA protease. Cells expressing Q688 produce higher ATP levels and ROS, promoting cell proliferation. Our results thus reveal an unexpected link between the phosphorylation-dependent regulation of the mitochondria mAAA protease affecting ROS production and several clinical phenotypes. Biology (General) Hsiao-Huei Chen verfasserin aut Ryan J. Mailloux verfasserin aut Takashi Tatsuta verfasserin aut Allen C.T. Teng verfasserin aut Ahmad B. Mahmoud verfasserin aut Tiffany Ho verfasserin aut Nicolas A.S. Stewart verfasserin aut Peter Rippstein verfasserin aut Mary Ellen Harper verfasserin aut Robert Roberts verfasserin aut Christina Willenborg verfasserin aut Jeanette Erdmann verfasserin aut Annalisa Pastore verfasserin aut Heidi M. McBride verfasserin aut Thomas Langer verfasserin aut Alexandre F.R. Stewart verfasserin aut In Cell Reports Elsevier, 2015 7(2014), 3, Seite 834-847 (DE-627)684964562 (DE-600)2649101-1 22111247 nnns volume:7 year:2014 number:3 pages:834-847 https://doi.org/10.1016/j.celrep.2014.03.051 kostenfrei https://doaj.org/article/aebe476dd1c04886a50974f3c1065d37 kostenfrei http://www.sciencedirect.com/science/article/pii/S2211124714002484 kostenfrei https://doaj.org/toc/2211-1247 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2110 GBV_ILN_2112 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4393 GBV_ILN_4700 AR 7 2014 3 834-847
allfields_unstemmed	10.1016/j.celrep.2014.03.051 doi (DE-627)DOAJ001489925 (DE-599)DOAJaebe476dd1c04886a50974f3c1065d37 DE-627 ger DE-627 rakwb eng QH301-705.5 Naif A.M. Almontashiri verfasserin aut SPG7 Variant Escapes Phosphorylation-Regulated Processing by AFG3L2, Elevates Mitochondrial ROS, and Is Associated with Multiple Clinical Phenotypes 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Mitochondrial production of reactive oxygen species (ROS) affects many processes in health and disease. SPG7 assembles with AFG3L2 into the mAAA protease at the inner membrane of mitochondria, degrades damaged proteins, and regulates the synthesis of mitochondrial ribosomes. SPG7 is cleaved and activated by AFG3L2 upon assembly. A variant in SPG7 that replaces arginine 688 with glutamine (Q688) is associated with several phenotypes, including toxicity of chemotherapeutic agents, type 2 diabetes mellitus, and (as reported here) coronary artery disease. We demonstrate that SPG7 processing is regulated by tyrosine phosphorylation of AFG3L2. Carriers of Q688 bypass this regulation and constitutively process and activate SPG7 mAAA protease. Cells expressing Q688 produce higher ATP levels and ROS, promoting cell proliferation. Our results thus reveal an unexpected link between the phosphorylation-dependent regulation of the mitochondria mAAA protease affecting ROS production and several clinical phenotypes. Biology (General) Hsiao-Huei Chen verfasserin aut Ryan J. Mailloux verfasserin aut Takashi Tatsuta verfasserin aut Allen C.T. Teng verfasserin aut Ahmad B. Mahmoud verfasserin aut Tiffany Ho verfasserin aut Nicolas A.S. Stewart verfasserin aut Peter Rippstein verfasserin aut Mary Ellen Harper verfasserin aut Robert Roberts verfasserin aut Christina Willenborg verfasserin aut Jeanette Erdmann verfasserin aut Annalisa Pastore verfasserin aut Heidi M. McBride verfasserin aut Thomas Langer verfasserin aut Alexandre F.R. Stewart verfasserin aut In Cell Reports Elsevier, 2015 7(2014), 3, Seite 834-847 (DE-627)684964562 (DE-600)2649101-1 22111247 nnns volume:7 year:2014 number:3 pages:834-847 https://doi.org/10.1016/j.celrep.2014.03.051 kostenfrei https://doaj.org/article/aebe476dd1c04886a50974f3c1065d37 kostenfrei http://www.sciencedirect.com/science/article/pii/S2211124714002484 kostenfrei https://doaj.org/toc/2211-1247 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2110 GBV_ILN_2112 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4393 GBV_ILN_4700 AR 7 2014 3 834-847
allfieldsGer	10.1016/j.celrep.2014.03.051 doi (DE-627)DOAJ001489925 (DE-599)DOAJaebe476dd1c04886a50974f3c1065d37 DE-627 ger DE-627 rakwb eng QH301-705.5 Naif A.M. Almontashiri verfasserin aut SPG7 Variant Escapes Phosphorylation-Regulated Processing by AFG3L2, Elevates Mitochondrial ROS, and Is Associated with Multiple Clinical Phenotypes 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Mitochondrial production of reactive oxygen species (ROS) affects many processes in health and disease. SPG7 assembles with AFG3L2 into the mAAA protease at the inner membrane of mitochondria, degrades damaged proteins, and regulates the synthesis of mitochondrial ribosomes. SPG7 is cleaved and activated by AFG3L2 upon assembly. A variant in SPG7 that replaces arginine 688 with glutamine (Q688) is associated with several phenotypes, including toxicity of chemotherapeutic agents, type 2 diabetes mellitus, and (as reported here) coronary artery disease. We demonstrate that SPG7 processing is regulated by tyrosine phosphorylation of AFG3L2. Carriers of Q688 bypass this regulation and constitutively process and activate SPG7 mAAA protease. Cells expressing Q688 produce higher ATP levels and ROS, promoting cell proliferation. Our results thus reveal an unexpected link between the phosphorylation-dependent regulation of the mitochondria mAAA protease affecting ROS production and several clinical phenotypes. Biology (General) Hsiao-Huei Chen verfasserin aut Ryan J. Mailloux verfasserin aut Takashi Tatsuta verfasserin aut Allen C.T. Teng verfasserin aut Ahmad B. Mahmoud verfasserin aut Tiffany Ho verfasserin aut Nicolas A.S. Stewart verfasserin aut Peter Rippstein verfasserin aut Mary Ellen Harper verfasserin aut Robert Roberts verfasserin aut Christina Willenborg verfasserin aut Jeanette Erdmann verfasserin aut Annalisa Pastore verfasserin aut Heidi M. McBride verfasserin aut Thomas Langer verfasserin aut Alexandre F.R. Stewart verfasserin aut In Cell Reports Elsevier, 2015 7(2014), 3, Seite 834-847 (DE-627)684964562 (DE-600)2649101-1 22111247 nnns volume:7 year:2014 number:3 pages:834-847 https://doi.org/10.1016/j.celrep.2014.03.051 kostenfrei https://doaj.org/article/aebe476dd1c04886a50974f3c1065d37 kostenfrei http://www.sciencedirect.com/science/article/pii/S2211124714002484 kostenfrei https://doaj.org/toc/2211-1247 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2110 GBV_ILN_2112 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4393 GBV_ILN_4700 AR 7 2014 3 834-847
allfieldsSound	10.1016/j.celrep.2014.03.051 doi (DE-627)DOAJ001489925 (DE-599)DOAJaebe476dd1c04886a50974f3c1065d37 DE-627 ger DE-627 rakwb eng QH301-705.5 Naif A.M. Almontashiri verfasserin aut SPG7 Variant Escapes Phosphorylation-Regulated Processing by AFG3L2, Elevates Mitochondrial ROS, and Is Associated with Multiple Clinical Phenotypes 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Mitochondrial production of reactive oxygen species (ROS) affects many processes in health and disease. SPG7 assembles with AFG3L2 into the mAAA protease at the inner membrane of mitochondria, degrades damaged proteins, and regulates the synthesis of mitochondrial ribosomes. SPG7 is cleaved and activated by AFG3L2 upon assembly. A variant in SPG7 that replaces arginine 688 with glutamine (Q688) is associated with several phenotypes, including toxicity of chemotherapeutic agents, type 2 diabetes mellitus, and (as reported here) coronary artery disease. We demonstrate that SPG7 processing is regulated by tyrosine phosphorylation of AFG3L2. Carriers of Q688 bypass this regulation and constitutively process and activate SPG7 mAAA protease. Cells expressing Q688 produce higher ATP levels and ROS, promoting cell proliferation. Our results thus reveal an unexpected link between the phosphorylation-dependent regulation of the mitochondria mAAA protease affecting ROS production and several clinical phenotypes. Biology (General) Hsiao-Huei Chen verfasserin aut Ryan J. Mailloux verfasserin aut Takashi Tatsuta verfasserin aut Allen C.T. Teng verfasserin aut Ahmad B. Mahmoud verfasserin aut Tiffany Ho verfasserin aut Nicolas A.S. Stewart verfasserin aut Peter Rippstein verfasserin aut Mary Ellen Harper verfasserin aut Robert Roberts verfasserin aut Christina Willenborg verfasserin aut Jeanette Erdmann verfasserin aut Annalisa Pastore verfasserin aut Heidi M. McBride verfasserin aut Thomas Langer verfasserin aut Alexandre F.R. Stewart verfasserin aut In Cell Reports Elsevier, 2015 7(2014), 3, Seite 834-847 (DE-627)684964562 (DE-600)2649101-1 22111247 nnns volume:7 year:2014 number:3 pages:834-847 https://doi.org/10.1016/j.celrep.2014.03.051 kostenfrei https://doaj.org/article/aebe476dd1c04886a50974f3c1065d37 kostenfrei http://www.sciencedirect.com/science/article/pii/S2211124714002484 kostenfrei https://doaj.org/toc/2211-1247 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2110 GBV_ILN_2112 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4393 GBV_ILN_4700 AR 7 2014 3 834-847
language	English
source	In Cell Reports 7(2014), 3, Seite 834-847 volume:7 year:2014 number:3 pages:834-847
sourceStr	In Cell Reports 7(2014), 3, Seite 834-847 volume:7 year:2014 number:3 pages:834-847
format_phy_str_mv	Article
institution	findex.gbv.de
topic_facet	Biology (General)
isfreeaccess_bool	true
container_title	Cell Reports
authorswithroles_txt_mv	Naif A.M. Almontashiri @@aut@@ Hsiao-Huei Chen @@aut@@ Ryan J. Mailloux @@aut@@ Takashi Tatsuta @@aut@@ Allen C.T. Teng @@aut@@ Ahmad B. Mahmoud @@aut@@ Tiffany Ho @@aut@@ Nicolas A.S. Stewart @@aut@@ Peter Rippstein @@aut@@ Mary Ellen Harper @@aut@@ Robert Roberts @@aut@@ Christina Willenborg @@aut@@ Jeanette Erdmann @@aut@@ Annalisa Pastore @@aut@@ Heidi M. McBride @@aut@@ Thomas Langer @@aut@@ Alexandre F.R. Stewart @@aut@@
publishDateDaySort_date	2014-01-01T00:00:00Z
hierarchy_top_id	684964562
id	DOAJ001489925
language_de	englisch
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callnumber-first	Q - Science
author	Naif A.M. Almontashiri
spellingShingle	Naif A.M. Almontashiri misc QH301-705.5 misc Biology (General) SPG7 Variant Escapes Phosphorylation-Regulated Processing by AFG3L2, Elevates Mitochondrial ROS, and Is Associated with Multiple Clinical Phenotypes
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topic_title	QH301-705.5 SPG7 Variant Escapes Phosphorylation-Regulated Processing by AFG3L2, Elevates Mitochondrial ROS, and Is Associated with Multiple Clinical Phenotypes
topic	misc QH301-705.5 misc Biology (General)
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title	SPG7 Variant Escapes Phosphorylation-Regulated Processing by AFG3L2, Elevates Mitochondrial ROS, and Is Associated with Multiple Clinical Phenotypes
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title_full	SPG7 Variant Escapes Phosphorylation-Regulated Processing by AFG3L2, Elevates Mitochondrial ROS, and Is Associated with Multiple Clinical Phenotypes
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author_browse	Naif A.M. Almontashiri Hsiao-Huei Chen Ryan J. Mailloux Takashi Tatsuta Allen C.T. Teng Ahmad B. Mahmoud Tiffany Ho Nicolas A.S. Stewart Peter Rippstein Mary Ellen Harper Robert Roberts Christina Willenborg Jeanette Erdmann Annalisa Pastore Heidi M. McBride Thomas Langer Alexandre F.R. Stewart
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title_sort	spg7 variant escapes phosphorylation-regulated processing by afg3l2, elevates mitochondrial ros, and is associated with multiple clinical phenotypes
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title_auth	SPG7 Variant Escapes Phosphorylation-Regulated Processing by AFG3L2, Elevates Mitochondrial ROS, and Is Associated with Multiple Clinical Phenotypes
abstract	Mitochondrial production of reactive oxygen species (ROS) affects many processes in health and disease. SPG7 assembles with AFG3L2 into the mAAA protease at the inner membrane of mitochondria, degrades damaged proteins, and regulates the synthesis of mitochondrial ribosomes. SPG7 is cleaved and activated by AFG3L2 upon assembly. A variant in SPG7 that replaces arginine 688 with glutamine (Q688) is associated with several phenotypes, including toxicity of chemotherapeutic agents, type 2 diabetes mellitus, and (as reported here) coronary artery disease. We demonstrate that SPG7 processing is regulated by tyrosine phosphorylation of AFG3L2. Carriers of Q688 bypass this regulation and constitutively process and activate SPG7 mAAA protease. Cells expressing Q688 produce higher ATP levels and ROS, promoting cell proliferation. Our results thus reveal an unexpected link between the phosphorylation-dependent regulation of the mitochondria mAAA protease affecting ROS production and several clinical phenotypes.
abstractGer	Mitochondrial production of reactive oxygen species (ROS) affects many processes in health and disease. SPG7 assembles with AFG3L2 into the mAAA protease at the inner membrane of mitochondria, degrades damaged proteins, and regulates the synthesis of mitochondrial ribosomes. SPG7 is cleaved and activated by AFG3L2 upon assembly. A variant in SPG7 that replaces arginine 688 with glutamine (Q688) is associated with several phenotypes, including toxicity of chemotherapeutic agents, type 2 diabetes mellitus, and (as reported here) coronary artery disease. We demonstrate that SPG7 processing is regulated by tyrosine phosphorylation of AFG3L2. Carriers of Q688 bypass this regulation and constitutively process and activate SPG7 mAAA protease. Cells expressing Q688 produce higher ATP levels and ROS, promoting cell proliferation. Our results thus reveal an unexpected link between the phosphorylation-dependent regulation of the mitochondria mAAA protease affecting ROS production and several clinical phenotypes.
abstract_unstemmed	Mitochondrial production of reactive oxygen species (ROS) affects many processes in health and disease. SPG7 assembles with AFG3L2 into the mAAA protease at the inner membrane of mitochondria, degrades damaged proteins, and regulates the synthesis of mitochondrial ribosomes. SPG7 is cleaved and activated by AFG3L2 upon assembly. A variant in SPG7 that replaces arginine 688 with glutamine (Q688) is associated with several phenotypes, including toxicity of chemotherapeutic agents, type 2 diabetes mellitus, and (as reported here) coronary artery disease. We demonstrate that SPG7 processing is regulated by tyrosine phosphorylation of AFG3L2. Carriers of Q688 bypass this regulation and constitutively process and activate SPG7 mAAA protease. Cells expressing Q688 produce higher ATP levels and ROS, promoting cell proliferation. Our results thus reveal an unexpected link between the phosphorylation-dependent regulation of the mitochondria mAAA protease affecting ROS production and several clinical phenotypes.
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title_short	SPG7 Variant Escapes Phosphorylation-Regulated Processing by AFG3L2, Elevates Mitochondrial ROS, and Is Associated with Multiple Clinical Phenotypes
url	https://doi.org/10.1016/j.celrep.2014.03.051 https://doaj.org/article/aebe476dd1c04886a50974f3c1065d37 http://www.sciencedirect.com/science/article/pii/S2211124714002484 https://doaj.org/toc/2211-1247
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up_date	2024-07-03T20:44:31.739Z
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SPG7 Variant Escapes Phosphorylation-Regulated Processing by AFG3L2, Elevates Mitochondrial ROS, and Is Associated with Multiple Clinical Phenotypes

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