Inborn Errors of Long-Chain Fatty Acid β-Oxidation Link Neural Stem Cell Self-Renewal to Autism

Summary: Inborn errors of metabolism (IEMs) occur with high incidence in human populations. Especially prevalent among these are inborn deficiencies in fatty acid β-oxidation (FAO), which are clinically associated with developmental neuropsychiatric disorders, including autism. We now report that ne...
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Autor*in:	Zhigang Xie [verfasserIn] Albert Jones [verfasserIn] Jude T. Deeney [verfasserIn] Seong Kwon Hur [verfasserIn] Vytas A. Bankaitis [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2016

Übergeordnetes Werk:	In: Cell Reports - Elsevier, 2015, 14(2016), 5, Seite 991-999
Übergeordnetes Werk:	volume:14 ; year:2016 ; number:5 ; pages:991-999

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc

DOI / URN:	10.1016/j.celrep.2016.01.004

Katalog-ID:	DOAJ034127992

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allfields	10.1016/j.celrep.2016.01.004 doi (DE-627)DOAJ034127992 (DE-599)DOAJ677d4391706e432687a66dd2704b9214 DE-627 ger DE-627 rakwb eng QH301-705.5 Zhigang Xie verfasserin aut Inborn Errors of Long-Chain Fatty Acid β-Oxidation Link Neural Stem Cell Self-Renewal to Autism 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Summary: Inborn errors of metabolism (IEMs) occur with high incidence in human populations. Especially prevalent among these are inborn deficiencies in fatty acid β-oxidation (FAO), which are clinically associated with developmental neuropsychiatric disorders, including autism. We now report that neural stem cell (NSC)-autonomous insufficiencies in the activity of TMLHE (an autism risk factor that supports long-chain FAO by catalyzing carnitine biosynthesis), of CPT1A (an enzyme required for long-chain FAO transport into mitochondria), or of fatty acid mobilization from lipid droplets reduced NSC pools in the mouse embryonic neocortex. Lineage tracing experiments demonstrated that reduced flux through the FAO pathway potentiated NSC symmetric differentiating divisions at the expense of self-renewing stem cell division modes. The collective data reveal a key role for FAO in controlling NSC-to-IPC transition in the mammalian embryonic brain and suggest NSC self renewal as a cellular mechanism underlying the association between IEMs and autism. : The mechanisms underlying the association between inborn errors of fatty acid metabolism and developmental brain disorders such as autism remain unclear. Xie et al. find that TMLHE, a carnitine biosynthesis enzyme, and carnitine-dependent long-chain fatty acid β-oxidation control the neural stem cell pool during neocortical development by maintaining self-renewing divisions. Biology (General) Albert Jones verfasserin aut Jude T. Deeney verfasserin aut Seong Kwon Hur verfasserin aut Vytas A. Bankaitis verfasserin aut In Cell Reports Elsevier, 2015 14(2016), 5, Seite 991-999 (DE-627)684964562 (DE-600)2649101-1 22111247 nnns volume:14 year:2016 number:5 pages:991-999 https://doi.org/10.1016/j.celrep.2016.01.004 kostenfrei https://doaj.org/article/677d4391706e432687a66dd2704b9214 kostenfrei http://www.sciencedirect.com/science/article/pii/S2211124716000085 kostenfrei https://doaj.org/toc/2211-1247 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ 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 14 2016 5 991-999
spelling	10.1016/j.celrep.2016.01.004 doi (DE-627)DOAJ034127992 (DE-599)DOAJ677d4391706e432687a66dd2704b9214 DE-627 ger DE-627 rakwb eng QH301-705.5 Zhigang Xie verfasserin aut Inborn Errors of Long-Chain Fatty Acid β-Oxidation Link Neural Stem Cell Self-Renewal to Autism 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Summary: Inborn errors of metabolism (IEMs) occur with high incidence in human populations. Especially prevalent among these are inborn deficiencies in fatty acid β-oxidation (FAO), which are clinically associated with developmental neuropsychiatric disorders, including autism. We now report that neural stem cell (NSC)-autonomous insufficiencies in the activity of TMLHE (an autism risk factor that supports long-chain FAO by catalyzing carnitine biosynthesis), of CPT1A (an enzyme required for long-chain FAO transport into mitochondria), or of fatty acid mobilization from lipid droplets reduced NSC pools in the mouse embryonic neocortex. Lineage tracing experiments demonstrated that reduced flux through the FAO pathway potentiated NSC symmetric differentiating divisions at the expense of self-renewing stem cell division modes. The collective data reveal a key role for FAO in controlling NSC-to-IPC transition in the mammalian embryonic brain and suggest NSC self renewal as a cellular mechanism underlying the association between IEMs and autism. : The mechanisms underlying the association between inborn errors of fatty acid metabolism and developmental brain disorders such as autism remain unclear. Xie et al. find that TMLHE, a carnitine biosynthesis enzyme, and carnitine-dependent long-chain fatty acid β-oxidation control the neural stem cell pool during neocortical development by maintaining self-renewing divisions. Biology (General) Albert Jones verfasserin aut Jude T. Deeney verfasserin aut Seong Kwon Hur verfasserin aut Vytas A. Bankaitis verfasserin aut In Cell Reports Elsevier, 2015 14(2016), 5, Seite 991-999 (DE-627)684964562 (DE-600)2649101-1 22111247 nnns volume:14 year:2016 number:5 pages:991-999 https://doi.org/10.1016/j.celrep.2016.01.004 kostenfrei https://doaj.org/article/677d4391706e432687a66dd2704b9214 kostenfrei http://www.sciencedirect.com/science/article/pii/S2211124716000085 kostenfrei https://doaj.org/toc/2211-1247 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ 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 14 2016 5 991-999
allfields_unstemmed	10.1016/j.celrep.2016.01.004 doi (DE-627)DOAJ034127992 (DE-599)DOAJ677d4391706e432687a66dd2704b9214 DE-627 ger DE-627 rakwb eng QH301-705.5 Zhigang Xie verfasserin aut Inborn Errors of Long-Chain Fatty Acid β-Oxidation Link Neural Stem Cell Self-Renewal to Autism 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Summary: Inborn errors of metabolism (IEMs) occur with high incidence in human populations. Especially prevalent among these are inborn deficiencies in fatty acid β-oxidation (FAO), which are clinically associated with developmental neuropsychiatric disorders, including autism. We now report that neural stem cell (NSC)-autonomous insufficiencies in the activity of TMLHE (an autism risk factor that supports long-chain FAO by catalyzing carnitine biosynthesis), of CPT1A (an enzyme required for long-chain FAO transport into mitochondria), or of fatty acid mobilization from lipid droplets reduced NSC pools in the mouse embryonic neocortex. Lineage tracing experiments demonstrated that reduced flux through the FAO pathway potentiated NSC symmetric differentiating divisions at the expense of self-renewing stem cell division modes. The collective data reveal a key role for FAO in controlling NSC-to-IPC transition in the mammalian embryonic brain and suggest NSC self renewal as a cellular mechanism underlying the association between IEMs and autism. : The mechanisms underlying the association between inborn errors of fatty acid metabolism and developmental brain disorders such as autism remain unclear. Xie et al. find that TMLHE, a carnitine biosynthesis enzyme, and carnitine-dependent long-chain fatty acid β-oxidation control the neural stem cell pool during neocortical development by maintaining self-renewing divisions. Biology (General) Albert Jones verfasserin aut Jude T. Deeney verfasserin aut Seong Kwon Hur verfasserin aut Vytas A. Bankaitis verfasserin aut In Cell Reports Elsevier, 2015 14(2016), 5, Seite 991-999 (DE-627)684964562 (DE-600)2649101-1 22111247 nnns volume:14 year:2016 number:5 pages:991-999 https://doi.org/10.1016/j.celrep.2016.01.004 kostenfrei https://doaj.org/article/677d4391706e432687a66dd2704b9214 kostenfrei http://www.sciencedirect.com/science/article/pii/S2211124716000085 kostenfrei https://doaj.org/toc/2211-1247 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ 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 14 2016 5 991-999
allfieldsGer	10.1016/j.celrep.2016.01.004 doi (DE-627)DOAJ034127992 (DE-599)DOAJ677d4391706e432687a66dd2704b9214 DE-627 ger DE-627 rakwb eng QH301-705.5 Zhigang Xie verfasserin aut Inborn Errors of Long-Chain Fatty Acid β-Oxidation Link Neural Stem Cell Self-Renewal to Autism 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Summary: Inborn errors of metabolism (IEMs) occur with high incidence in human populations. Especially prevalent among these are inborn deficiencies in fatty acid β-oxidation (FAO), which are clinically associated with developmental neuropsychiatric disorders, including autism. We now report that neural stem cell (NSC)-autonomous insufficiencies in the activity of TMLHE (an autism risk factor that supports long-chain FAO by catalyzing carnitine biosynthesis), of CPT1A (an enzyme required for long-chain FAO transport into mitochondria), or of fatty acid mobilization from lipid droplets reduced NSC pools in the mouse embryonic neocortex. Lineage tracing experiments demonstrated that reduced flux through the FAO pathway potentiated NSC symmetric differentiating divisions at the expense of self-renewing stem cell division modes. The collective data reveal a key role for FAO in controlling NSC-to-IPC transition in the mammalian embryonic brain and suggest NSC self renewal as a cellular mechanism underlying the association between IEMs and autism. : The mechanisms underlying the association between inborn errors of fatty acid metabolism and developmental brain disorders such as autism remain unclear. Xie et al. find that TMLHE, a carnitine biosynthesis enzyme, and carnitine-dependent long-chain fatty acid β-oxidation control the neural stem cell pool during neocortical development by maintaining self-renewing divisions. Biology (General) Albert Jones verfasserin aut Jude T. Deeney verfasserin aut Seong Kwon Hur verfasserin aut Vytas A. Bankaitis verfasserin aut In Cell Reports Elsevier, 2015 14(2016), 5, Seite 991-999 (DE-627)684964562 (DE-600)2649101-1 22111247 nnns volume:14 year:2016 number:5 pages:991-999 https://doi.org/10.1016/j.celrep.2016.01.004 kostenfrei https://doaj.org/article/677d4391706e432687a66dd2704b9214 kostenfrei http://www.sciencedirect.com/science/article/pii/S2211124716000085 kostenfrei https://doaj.org/toc/2211-1247 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ 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 14 2016 5 991-999
allfieldsSound	10.1016/j.celrep.2016.01.004 doi (DE-627)DOAJ034127992 (DE-599)DOAJ677d4391706e432687a66dd2704b9214 DE-627 ger DE-627 rakwb eng QH301-705.5 Zhigang Xie verfasserin aut Inborn Errors of Long-Chain Fatty Acid β-Oxidation Link Neural Stem Cell Self-Renewal to Autism 2016 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Summary: Inborn errors of metabolism (IEMs) occur with high incidence in human populations. Especially prevalent among these are inborn deficiencies in fatty acid β-oxidation (FAO), which are clinically associated with developmental neuropsychiatric disorders, including autism. We now report that neural stem cell (NSC)-autonomous insufficiencies in the activity of TMLHE (an autism risk factor that supports long-chain FAO by catalyzing carnitine biosynthesis), of CPT1A (an enzyme required for long-chain FAO transport into mitochondria), or of fatty acid mobilization from lipid droplets reduced NSC pools in the mouse embryonic neocortex. Lineage tracing experiments demonstrated that reduced flux through the FAO pathway potentiated NSC symmetric differentiating divisions at the expense of self-renewing stem cell division modes. The collective data reveal a key role for FAO in controlling NSC-to-IPC transition in the mammalian embryonic brain and suggest NSC self renewal as a cellular mechanism underlying the association between IEMs and autism. : The mechanisms underlying the association between inborn errors of fatty acid metabolism and developmental brain disorders such as autism remain unclear. Xie et al. find that TMLHE, a carnitine biosynthesis enzyme, and carnitine-dependent long-chain fatty acid β-oxidation control the neural stem cell pool during neocortical development by maintaining self-renewing divisions. Biology (General) Albert Jones verfasserin aut Jude T. Deeney verfasserin aut Seong Kwon Hur verfasserin aut Vytas A. Bankaitis verfasserin aut In Cell Reports Elsevier, 2015 14(2016), 5, Seite 991-999 (DE-627)684964562 (DE-600)2649101-1 22111247 nnns volume:14 year:2016 number:5 pages:991-999 https://doi.org/10.1016/j.celrep.2016.01.004 kostenfrei https://doaj.org/article/677d4391706e432687a66dd2704b9214 kostenfrei http://www.sciencedirect.com/science/article/pii/S2211124716000085 kostenfrei https://doaj.org/toc/2211-1247 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ 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 14 2016 5 991-999
language	English
source	In Cell Reports 14(2016), 5, Seite 991-999 volume:14 year:2016 number:5 pages:991-999
sourceStr	In Cell Reports 14(2016), 5, Seite 991-999 volume:14 year:2016 number:5 pages:991-999
format_phy_str_mv	Article
institution	findex.gbv.de
topic_facet	Biology (General)
isfreeaccess_bool	true
container_title	Cell Reports
authorswithroles_txt_mv	Zhigang Xie @@aut@@ Albert Jones @@aut@@ Jude T. Deeney @@aut@@ Seong Kwon Hur @@aut@@ Vytas A. Bankaitis @@aut@@
publishDateDaySort_date	2016-01-01T00:00:00Z
hierarchy_top_id	684964562
id	DOAJ034127992
language_de	englisch
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callnumber-first	Q - Science
author	Zhigang Xie
spellingShingle	Zhigang Xie misc QH301-705.5 misc Biology (General) Inborn Errors of Long-Chain Fatty Acid β-Oxidation Link Neural Stem Cell Self-Renewal to Autism
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topic_title	QH301-705.5 Inborn Errors of Long-Chain Fatty Acid β-Oxidation Link Neural Stem Cell Self-Renewal to Autism
topic	misc QH301-705.5 misc Biology (General)
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title	Inborn Errors of Long-Chain Fatty Acid β-Oxidation Link Neural Stem Cell Self-Renewal to Autism
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title_full	Inborn Errors of Long-Chain Fatty Acid β-Oxidation Link Neural Stem Cell Self-Renewal to Autism
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author_browse	Zhigang Xie Albert Jones Jude T. Deeney Seong Kwon Hur Vytas A. Bankaitis
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title_sort	inborn errors of long-chain fatty acid β-oxidation link neural stem cell self-renewal to autism
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title_auth	Inborn Errors of Long-Chain Fatty Acid β-Oxidation Link Neural Stem Cell Self-Renewal to Autism
abstract	Summary: Inborn errors of metabolism (IEMs) occur with high incidence in human populations. Especially prevalent among these are inborn deficiencies in fatty acid β-oxidation (FAO), which are clinically associated with developmental neuropsychiatric disorders, including autism. We now report that neural stem cell (NSC)-autonomous insufficiencies in the activity of TMLHE (an autism risk factor that supports long-chain FAO by catalyzing carnitine biosynthesis), of CPT1A (an enzyme required for long-chain FAO transport into mitochondria), or of fatty acid mobilization from lipid droplets reduced NSC pools in the mouse embryonic neocortex. Lineage tracing experiments demonstrated that reduced flux through the FAO pathway potentiated NSC symmetric differentiating divisions at the expense of self-renewing stem cell division modes. The collective data reveal a key role for FAO in controlling NSC-to-IPC transition in the mammalian embryonic brain and suggest NSC self renewal as a cellular mechanism underlying the association between IEMs and autism. : The mechanisms underlying the association between inborn errors of fatty acid metabolism and developmental brain disorders such as autism remain unclear. Xie et al. find that TMLHE, a carnitine biosynthesis enzyme, and carnitine-dependent long-chain fatty acid β-oxidation control the neural stem cell pool during neocortical development by maintaining self-renewing divisions.
abstractGer	Summary: Inborn errors of metabolism (IEMs) occur with high incidence in human populations. Especially prevalent among these are inborn deficiencies in fatty acid β-oxidation (FAO), which are clinically associated with developmental neuropsychiatric disorders, including autism. We now report that neural stem cell (NSC)-autonomous insufficiencies in the activity of TMLHE (an autism risk factor that supports long-chain FAO by catalyzing carnitine biosynthesis), of CPT1A (an enzyme required for long-chain FAO transport into mitochondria), or of fatty acid mobilization from lipid droplets reduced NSC pools in the mouse embryonic neocortex. Lineage tracing experiments demonstrated that reduced flux through the FAO pathway potentiated NSC symmetric differentiating divisions at the expense of self-renewing stem cell division modes. The collective data reveal a key role for FAO in controlling NSC-to-IPC transition in the mammalian embryonic brain and suggest NSC self renewal as a cellular mechanism underlying the association between IEMs and autism. : The mechanisms underlying the association between inborn errors of fatty acid metabolism and developmental brain disorders such as autism remain unclear. Xie et al. find that TMLHE, a carnitine biosynthesis enzyme, and carnitine-dependent long-chain fatty acid β-oxidation control the neural stem cell pool during neocortical development by maintaining self-renewing divisions.
abstract_unstemmed	Summary: Inborn errors of metabolism (IEMs) occur with high incidence in human populations. Especially prevalent among these are inborn deficiencies in fatty acid β-oxidation (FAO), which are clinically associated with developmental neuropsychiatric disorders, including autism. We now report that neural stem cell (NSC)-autonomous insufficiencies in the activity of TMLHE (an autism risk factor that supports long-chain FAO by catalyzing carnitine biosynthesis), of CPT1A (an enzyme required for long-chain FAO transport into mitochondria), or of fatty acid mobilization from lipid droplets reduced NSC pools in the mouse embryonic neocortex. Lineage tracing experiments demonstrated that reduced flux through the FAO pathway potentiated NSC symmetric differentiating divisions at the expense of self-renewing stem cell division modes. The collective data reveal a key role for FAO in controlling NSC-to-IPC transition in the mammalian embryonic brain and suggest NSC self renewal as a cellular mechanism underlying the association between IEMs and autism. : The mechanisms underlying the association between inborn errors of fatty acid metabolism and developmental brain disorders such as autism remain unclear. Xie et al. find that TMLHE, a carnitine biosynthesis enzyme, and carnitine-dependent long-chain fatty acid β-oxidation control the neural stem cell pool during neocortical development by maintaining self-renewing divisions.
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title_short	Inborn Errors of Long-Chain Fatty Acid β-Oxidation Link Neural Stem Cell Self-Renewal to Autism
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Inborn Errors of Long-Chain Fatty Acid β-Oxidation Link Neural Stem Cell Self-Renewal to Autism

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