Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway

Abstract Coenzyme Q (CoQ) is an electron acceptor for sulfide‐quinone reductase (SQR), the first enzyme of the hydrogen sulfide oxidation pathway. Here, we show that lack of CoQ in human skin fibroblasts causes impairment of hydrogen sulfide oxidation, proportional to the residual levels of CoQ. Bio...
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Autor*in:	Marcello Ziosi [verfasserIn] Ivano Di Meo [verfasserIn] Giulio Kleiner [verfasserIn] Xing‐Huang Gao [verfasserIn] Emanuele Barca [verfasserIn] Maria J Sanchez‐Quintero [verfasserIn] Saba Tadesse [verfasserIn] Hongfeng Jiang [verfasserIn] Changhong Qiao [verfasserIn] Richard J Rodenburg [verfasserIn] Emmanuel Scalais [verfasserIn] Markus Schuelke [verfasserIn] Belinda Willard [verfasserIn] Maria Hatzoglou [verfasserIn] Valeria Tiranti [verfasserIn] Catarina M Quinzii [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2017

Schlagwörter:	coenzyme Q CoQ10 Pdss2 SQR sulfides

Übergeordnetes Werk:	In: EMBO Molecular Medicine - Wiley, 2012, 9(2017), 1, Seite 96-111
Übergeordnetes Werk:	volume:9 ; year:2017 ; number:1 ; pages:96-111

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc Journal toc

DOI / URN:	10.15252/emmm.201606356

Katalog-ID:	DOAJ098478931

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520			\|a Abstract Coenzyme Q (CoQ) is an electron acceptor for sulfide‐quinone reductase (SQR), the first enzyme of the hydrogen sulfide oxidation pathway. Here, we show that lack of CoQ in human skin fibroblasts causes impairment of hydrogen sulfide oxidation, proportional to the residual levels of CoQ. Biochemical and molecular abnormalities are rescued by CoQ supplementation in vitro and recapitulated by pharmacological inhibition of CoQ biosynthesis in skin fibroblasts and ADCK3 depletion in HeLa cells. Kidneys of Pdss2kd/kd mice, which only have ~15% residual CoQ concentrations and are clinically affected, showed (i) reduced protein levels of SQR and downstream enzymes, (ii) accumulation of hydrogen sulfides, and (iii) glutathione depletion. These abnormalities were not present in brain, which maintains ~30% residual CoQ and is clinically unaffected. In Pdss2kd/kd mice, we also observed low levels of plasma and urine thiosulfate and increased blood C4‐C6 acylcarnitines. We propose that impairment of the sulfide oxidation pathway induced by decreased levels of CoQ causes accumulation of sulfides and consequent inhibition of short‐chain acyl‐CoA dehydrogenase and glutathione depletion, which contributes to increased oxidative stress and kidney failure.
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700	0		\|a Xing‐Huang Gao \|e verfasserin \|4 aut
700	0		\|a Emanuele Barca \|e verfasserin \|4 aut
700	0		\|a Maria J Sanchez‐Quintero \|e verfasserin \|4 aut
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700	0		\|a Hongfeng Jiang \|e verfasserin \|4 aut
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700	0		\|a Emmanuel Scalais \|e verfasserin \|4 aut
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700	0		\|a Belinda Willard \|e verfasserin \|4 aut
700	0		\|a Maria Hatzoglou \|e verfasserin \|4 aut
700	0		\|a Valeria Tiranti \|e verfasserin \|4 aut
700	0		\|a Catarina M Quinzii \|e verfasserin \|4 aut
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author_variant	m z mz i d m idm g k gk x g xg e b eb m j s mjs s t st h j hj c q cq r j r rjr e s es m s ms b w bw m h mh v t vt c m q cmq
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allfields	10.15252/emmm.201606356 doi (DE-627)DOAJ098478931 (DE-599)DOAJc93d874d27cf40a7a84b8c58b34251f8 DE-627 ger DE-627 rakwb eng R5-920 QH426-470 Marcello Ziosi verfasserin aut Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway 2017 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Abstract Coenzyme Q (CoQ) is an electron acceptor for sulfide‐quinone reductase (SQR), the first enzyme of the hydrogen sulfide oxidation pathway. Here, we show that lack of CoQ in human skin fibroblasts causes impairment of hydrogen sulfide oxidation, proportional to the residual levels of CoQ. Biochemical and molecular abnormalities are rescued by CoQ supplementation in vitro and recapitulated by pharmacological inhibition of CoQ biosynthesis in skin fibroblasts and ADCK3 depletion in HeLa cells. Kidneys of Pdss2kd/kd mice, which only have ~15% residual CoQ concentrations and are clinically affected, showed (i) reduced protein levels of SQR and downstream enzymes, (ii) accumulation of hydrogen sulfides, and (iii) glutathione depletion. These abnormalities were not present in brain, which maintains ~30% residual CoQ and is clinically unaffected. In Pdss2kd/kd mice, we also observed low levels of plasma and urine thiosulfate and increased blood C4‐C6 acylcarnitines. We propose that impairment of the sulfide oxidation pathway induced by decreased levels of CoQ causes accumulation of sulfides and consequent inhibition of short‐chain acyl‐CoA dehydrogenase and glutathione depletion, which contributes to increased oxidative stress and kidney failure. coenzyme Q CoQ10 Pdss2 SQR sulfides Medicine (General) Genetics Ivano Di Meo verfasserin aut Giulio Kleiner verfasserin aut Xing‐Huang Gao verfasserin aut Emanuele Barca verfasserin aut Maria J Sanchez‐Quintero verfasserin aut Saba Tadesse verfasserin aut Hongfeng Jiang verfasserin aut Changhong Qiao verfasserin aut Richard J Rodenburg verfasserin aut Emmanuel Scalais verfasserin aut Markus Schuelke verfasserin aut Belinda Willard verfasserin aut Maria Hatzoglou verfasserin aut Valeria Tiranti verfasserin aut Catarina M Quinzii verfasserin aut In EMBO Molecular Medicine Wiley, 2012 9(2017), 1, Seite 96-111 (DE-627)594772761 (DE-600)2485479-7 17574684 nnns volume:9 year:2017 number:1 pages:96-111 https://doi.org/10.15252/emmm.201606356 kostenfrei https://doaj.org/article/c93d874d27cf40a7a84b8c58b34251f8 kostenfrei https://doi.org/10.15252/emmm.201606356 kostenfrei https://doaj.org/toc/1757-4676 Journal toc kostenfrei https://doaj.org/toc/1757-4684 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 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_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2232 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 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_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 9 2017 1 96-111
spelling	10.15252/emmm.201606356 doi (DE-627)DOAJ098478931 (DE-599)DOAJc93d874d27cf40a7a84b8c58b34251f8 DE-627 ger DE-627 rakwb eng R5-920 QH426-470 Marcello Ziosi verfasserin aut Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway 2017 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Abstract Coenzyme Q (CoQ) is an electron acceptor for sulfide‐quinone reductase (SQR), the first enzyme of the hydrogen sulfide oxidation pathway. Here, we show that lack of CoQ in human skin fibroblasts causes impairment of hydrogen sulfide oxidation, proportional to the residual levels of CoQ. Biochemical and molecular abnormalities are rescued by CoQ supplementation in vitro and recapitulated by pharmacological inhibition of CoQ biosynthesis in skin fibroblasts and ADCK3 depletion in HeLa cells. Kidneys of Pdss2kd/kd mice, which only have ~15% residual CoQ concentrations and are clinically affected, showed (i) reduced protein levels of SQR and downstream enzymes, (ii) accumulation of hydrogen sulfides, and (iii) glutathione depletion. These abnormalities were not present in brain, which maintains ~30% residual CoQ and is clinically unaffected. In Pdss2kd/kd mice, we also observed low levels of plasma and urine thiosulfate and increased blood C4‐C6 acylcarnitines. We propose that impairment of the sulfide oxidation pathway induced by decreased levels of CoQ causes accumulation of sulfides and consequent inhibition of short‐chain acyl‐CoA dehydrogenase and glutathione depletion, which contributes to increased oxidative stress and kidney failure. coenzyme Q CoQ10 Pdss2 SQR sulfides Medicine (General) Genetics Ivano Di Meo verfasserin aut Giulio Kleiner verfasserin aut Xing‐Huang Gao verfasserin aut Emanuele Barca verfasserin aut Maria J Sanchez‐Quintero verfasserin aut Saba Tadesse verfasserin aut Hongfeng Jiang verfasserin aut Changhong Qiao verfasserin aut Richard J Rodenburg verfasserin aut Emmanuel Scalais verfasserin aut Markus Schuelke verfasserin aut Belinda Willard verfasserin aut Maria Hatzoglou verfasserin aut Valeria Tiranti verfasserin aut Catarina M Quinzii verfasserin aut In EMBO Molecular Medicine Wiley, 2012 9(2017), 1, Seite 96-111 (DE-627)594772761 (DE-600)2485479-7 17574684 nnns volume:9 year:2017 number:1 pages:96-111 https://doi.org/10.15252/emmm.201606356 kostenfrei https://doaj.org/article/c93d874d27cf40a7a84b8c58b34251f8 kostenfrei https://doi.org/10.15252/emmm.201606356 kostenfrei https://doaj.org/toc/1757-4676 Journal toc kostenfrei https://doaj.org/toc/1757-4684 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 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_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2232 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 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_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 9 2017 1 96-111
allfields_unstemmed	10.15252/emmm.201606356 doi (DE-627)DOAJ098478931 (DE-599)DOAJc93d874d27cf40a7a84b8c58b34251f8 DE-627 ger DE-627 rakwb eng R5-920 QH426-470 Marcello Ziosi verfasserin aut Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway 2017 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Abstract Coenzyme Q (CoQ) is an electron acceptor for sulfide‐quinone reductase (SQR), the first enzyme of the hydrogen sulfide oxidation pathway. Here, we show that lack of CoQ in human skin fibroblasts causes impairment of hydrogen sulfide oxidation, proportional to the residual levels of CoQ. Biochemical and molecular abnormalities are rescued by CoQ supplementation in vitro and recapitulated by pharmacological inhibition of CoQ biosynthesis in skin fibroblasts and ADCK3 depletion in HeLa cells. Kidneys of Pdss2kd/kd mice, which only have ~15% residual CoQ concentrations and are clinically affected, showed (i) reduced protein levels of SQR and downstream enzymes, (ii) accumulation of hydrogen sulfides, and (iii) glutathione depletion. These abnormalities were not present in brain, which maintains ~30% residual CoQ and is clinically unaffected. In Pdss2kd/kd mice, we also observed low levels of plasma and urine thiosulfate and increased blood C4‐C6 acylcarnitines. We propose that impairment of the sulfide oxidation pathway induced by decreased levels of CoQ causes accumulation of sulfides and consequent inhibition of short‐chain acyl‐CoA dehydrogenase and glutathione depletion, which contributes to increased oxidative stress and kidney failure. coenzyme Q CoQ10 Pdss2 SQR sulfides Medicine (General) Genetics Ivano Di Meo verfasserin aut Giulio Kleiner verfasserin aut Xing‐Huang Gao verfasserin aut Emanuele Barca verfasserin aut Maria J Sanchez‐Quintero verfasserin aut Saba Tadesse verfasserin aut Hongfeng Jiang verfasserin aut Changhong Qiao verfasserin aut Richard J Rodenburg verfasserin aut Emmanuel Scalais verfasserin aut Markus Schuelke verfasserin aut Belinda Willard verfasserin aut Maria Hatzoglou verfasserin aut Valeria Tiranti verfasserin aut Catarina M Quinzii verfasserin aut In EMBO Molecular Medicine Wiley, 2012 9(2017), 1, Seite 96-111 (DE-627)594772761 (DE-600)2485479-7 17574684 nnns volume:9 year:2017 number:1 pages:96-111 https://doi.org/10.15252/emmm.201606356 kostenfrei https://doaj.org/article/c93d874d27cf40a7a84b8c58b34251f8 kostenfrei https://doi.org/10.15252/emmm.201606356 kostenfrei https://doaj.org/toc/1757-4676 Journal toc kostenfrei https://doaj.org/toc/1757-4684 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 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_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2232 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 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_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 9 2017 1 96-111
allfieldsGer	10.15252/emmm.201606356 doi (DE-627)DOAJ098478931 (DE-599)DOAJc93d874d27cf40a7a84b8c58b34251f8 DE-627 ger DE-627 rakwb eng R5-920 QH426-470 Marcello Ziosi verfasserin aut Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway 2017 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Abstract Coenzyme Q (CoQ) is an electron acceptor for sulfide‐quinone reductase (SQR), the first enzyme of the hydrogen sulfide oxidation pathway. Here, we show that lack of CoQ in human skin fibroblasts causes impairment of hydrogen sulfide oxidation, proportional to the residual levels of CoQ. Biochemical and molecular abnormalities are rescued by CoQ supplementation in vitro and recapitulated by pharmacological inhibition of CoQ biosynthesis in skin fibroblasts and ADCK3 depletion in HeLa cells. Kidneys of Pdss2kd/kd mice, which only have ~15% residual CoQ concentrations and are clinically affected, showed (i) reduced protein levels of SQR and downstream enzymes, (ii) accumulation of hydrogen sulfides, and (iii) glutathione depletion. These abnormalities were not present in brain, which maintains ~30% residual CoQ and is clinically unaffected. In Pdss2kd/kd mice, we also observed low levels of plasma and urine thiosulfate and increased blood C4‐C6 acylcarnitines. We propose that impairment of the sulfide oxidation pathway induced by decreased levels of CoQ causes accumulation of sulfides and consequent inhibition of short‐chain acyl‐CoA dehydrogenase and glutathione depletion, which contributes to increased oxidative stress and kidney failure. coenzyme Q CoQ10 Pdss2 SQR sulfides Medicine (General) Genetics Ivano Di Meo verfasserin aut Giulio Kleiner verfasserin aut Xing‐Huang Gao verfasserin aut Emanuele Barca verfasserin aut Maria J Sanchez‐Quintero verfasserin aut Saba Tadesse verfasserin aut Hongfeng Jiang verfasserin aut Changhong Qiao verfasserin aut Richard J Rodenburg verfasserin aut Emmanuel Scalais verfasserin aut Markus Schuelke verfasserin aut Belinda Willard verfasserin aut Maria Hatzoglou verfasserin aut Valeria Tiranti verfasserin aut Catarina M Quinzii verfasserin aut In EMBO Molecular Medicine Wiley, 2012 9(2017), 1, Seite 96-111 (DE-627)594772761 (DE-600)2485479-7 17574684 nnns volume:9 year:2017 number:1 pages:96-111 https://doi.org/10.15252/emmm.201606356 kostenfrei https://doaj.org/article/c93d874d27cf40a7a84b8c58b34251f8 kostenfrei https://doi.org/10.15252/emmm.201606356 kostenfrei https://doaj.org/toc/1757-4676 Journal toc kostenfrei https://doaj.org/toc/1757-4684 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 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_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2232 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 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_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 9 2017 1 96-111
allfieldsSound	10.15252/emmm.201606356 doi (DE-627)DOAJ098478931 (DE-599)DOAJc93d874d27cf40a7a84b8c58b34251f8 DE-627 ger DE-627 rakwb eng R5-920 QH426-470 Marcello Ziosi verfasserin aut Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway 2017 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Abstract Coenzyme Q (CoQ) is an electron acceptor for sulfide‐quinone reductase (SQR), the first enzyme of the hydrogen sulfide oxidation pathway. Here, we show that lack of CoQ in human skin fibroblasts causes impairment of hydrogen sulfide oxidation, proportional to the residual levels of CoQ. Biochemical and molecular abnormalities are rescued by CoQ supplementation in vitro and recapitulated by pharmacological inhibition of CoQ biosynthesis in skin fibroblasts and ADCK3 depletion in HeLa cells. Kidneys of Pdss2kd/kd mice, which only have ~15% residual CoQ concentrations and are clinically affected, showed (i) reduced protein levels of SQR and downstream enzymes, (ii) accumulation of hydrogen sulfides, and (iii) glutathione depletion. These abnormalities were not present in brain, which maintains ~30% residual CoQ and is clinically unaffected. In Pdss2kd/kd mice, we also observed low levels of plasma and urine thiosulfate and increased blood C4‐C6 acylcarnitines. We propose that impairment of the sulfide oxidation pathway induced by decreased levels of CoQ causes accumulation of sulfides and consequent inhibition of short‐chain acyl‐CoA dehydrogenase and glutathione depletion, which contributes to increased oxidative stress and kidney failure. coenzyme Q CoQ10 Pdss2 SQR sulfides Medicine (General) Genetics Ivano Di Meo verfasserin aut Giulio Kleiner verfasserin aut Xing‐Huang Gao verfasserin aut Emanuele Barca verfasserin aut Maria J Sanchez‐Quintero verfasserin aut Saba Tadesse verfasserin aut Hongfeng Jiang verfasserin aut Changhong Qiao verfasserin aut Richard J Rodenburg verfasserin aut Emmanuel Scalais verfasserin aut Markus Schuelke verfasserin aut Belinda Willard verfasserin aut Maria Hatzoglou verfasserin aut Valeria Tiranti verfasserin aut Catarina M Quinzii verfasserin aut In EMBO Molecular Medicine Wiley, 2012 9(2017), 1, Seite 96-111 (DE-627)594772761 (DE-600)2485479-7 17574684 nnns volume:9 year:2017 number:1 pages:96-111 https://doi.org/10.15252/emmm.201606356 kostenfrei https://doaj.org/article/c93d874d27cf40a7a84b8c58b34251f8 kostenfrei https://doi.org/10.15252/emmm.201606356 kostenfrei https://doaj.org/toc/1757-4676 Journal toc kostenfrei https://doaj.org/toc/1757-4684 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 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_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2232 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 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_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 9 2017 1 96-111
language	English
source	In EMBO Molecular Medicine 9(2017), 1, Seite 96-111 volume:9 year:2017 number:1 pages:96-111
sourceStr	In EMBO Molecular Medicine 9(2017), 1, Seite 96-111 volume:9 year:2017 number:1 pages:96-111
format_phy_str_mv	Article
institution	findex.gbv.de
topic_facet	coenzyme Q CoQ10 Pdss2 SQR sulfides Medicine (General) Genetics
isfreeaccess_bool	true
container_title	EMBO Molecular Medicine
authorswithroles_txt_mv	Marcello Ziosi @@aut@@ Ivano Di Meo @@aut@@ Giulio Kleiner @@aut@@ Xing‐Huang Gao @@aut@@ Emanuele Barca @@aut@@ Maria J Sanchez‐Quintero @@aut@@ Saba Tadesse @@aut@@ Hongfeng Jiang @@aut@@ Changhong Qiao @@aut@@ Richard J Rodenburg @@aut@@ Emmanuel Scalais @@aut@@ Markus Schuelke @@aut@@ Belinda Willard @@aut@@ Maria Hatzoglou @@aut@@ Valeria Tiranti @@aut@@ Catarina M Quinzii @@aut@@
publishDateDaySort_date	2017-01-01T00:00:00Z
hierarchy_top_id	594772761
id	DOAJ098478931
language_de	englisch
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callnumber-first	R - Medicine
author	Marcello Ziosi
spellingShingle	Marcello Ziosi misc R5-920 misc QH426-470 misc coenzyme Q misc CoQ10 misc Pdss2 misc SQR misc sulfides misc Medicine (General) misc Genetics Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway
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topic_title	R5-920 QH426-470 Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway coenzyme Q CoQ10 Pdss2 SQR sulfides
topic	misc R5-920 misc QH426-470 misc coenzyme Q misc CoQ10 misc Pdss2 misc SQR misc sulfides misc Medicine (General) misc Genetics
topic_unstemmed	misc R5-920 misc QH426-470 misc coenzyme Q misc CoQ10 misc Pdss2 misc SQR misc sulfides misc Medicine (General) misc Genetics
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title	Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway
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title_full	Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway
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author_browse	Marcello Ziosi Ivano Di Meo Giulio Kleiner Xing‐Huang Gao Emanuele Barca Maria J Sanchez‐Quintero Saba Tadesse Hongfeng Jiang Changhong Qiao Richard J Rodenburg Emmanuel Scalais Markus Schuelke Belinda Willard Maria Hatzoglou Valeria Tiranti Catarina M Quinzii
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title_sort	coenzyme q deficiency causes impairment of the sulfide oxidation pathway
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title_auth	Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway
abstract	Abstract Coenzyme Q (CoQ) is an electron acceptor for sulfide‐quinone reductase (SQR), the first enzyme of the hydrogen sulfide oxidation pathway. Here, we show that lack of CoQ in human skin fibroblasts causes impairment of hydrogen sulfide oxidation, proportional to the residual levels of CoQ. Biochemical and molecular abnormalities are rescued by CoQ supplementation in vitro and recapitulated by pharmacological inhibition of CoQ biosynthesis in skin fibroblasts and ADCK3 depletion in HeLa cells. Kidneys of Pdss2kd/kd mice, which only have ~15% residual CoQ concentrations and are clinically affected, showed (i) reduced protein levels of SQR and downstream enzymes, (ii) accumulation of hydrogen sulfides, and (iii) glutathione depletion. These abnormalities were not present in brain, which maintains ~30% residual CoQ and is clinically unaffected. In Pdss2kd/kd mice, we also observed low levels of plasma and urine thiosulfate and increased blood C4‐C6 acylcarnitines. We propose that impairment of the sulfide oxidation pathway induced by decreased levels of CoQ causes accumulation of sulfides and consequent inhibition of short‐chain acyl‐CoA dehydrogenase and glutathione depletion, which contributes to increased oxidative stress and kidney failure.
abstractGer	Abstract Coenzyme Q (CoQ) is an electron acceptor for sulfide‐quinone reductase (SQR), the first enzyme of the hydrogen sulfide oxidation pathway. Here, we show that lack of CoQ in human skin fibroblasts causes impairment of hydrogen sulfide oxidation, proportional to the residual levels of CoQ. Biochemical and molecular abnormalities are rescued by CoQ supplementation in vitro and recapitulated by pharmacological inhibition of CoQ biosynthesis in skin fibroblasts and ADCK3 depletion in HeLa cells. Kidneys of Pdss2kd/kd mice, which only have ~15% residual CoQ concentrations and are clinically affected, showed (i) reduced protein levels of SQR and downstream enzymes, (ii) accumulation of hydrogen sulfides, and (iii) glutathione depletion. These abnormalities were not present in brain, which maintains ~30% residual CoQ and is clinically unaffected. In Pdss2kd/kd mice, we also observed low levels of plasma and urine thiosulfate and increased blood C4‐C6 acylcarnitines. We propose that impairment of the sulfide oxidation pathway induced by decreased levels of CoQ causes accumulation of sulfides and consequent inhibition of short‐chain acyl‐CoA dehydrogenase and glutathione depletion, which contributes to increased oxidative stress and kidney failure.
abstract_unstemmed	Abstract Coenzyme Q (CoQ) is an electron acceptor for sulfide‐quinone reductase (SQR), the first enzyme of the hydrogen sulfide oxidation pathway. Here, we show that lack of CoQ in human skin fibroblasts causes impairment of hydrogen sulfide oxidation, proportional to the residual levels of CoQ. Biochemical and molecular abnormalities are rescued by CoQ supplementation in vitro and recapitulated by pharmacological inhibition of CoQ biosynthesis in skin fibroblasts and ADCK3 depletion in HeLa cells. Kidneys of Pdss2kd/kd mice, which only have ~15% residual CoQ concentrations and are clinically affected, showed (i) reduced protein levels of SQR and downstream enzymes, (ii) accumulation of hydrogen sulfides, and (iii) glutathione depletion. These abnormalities were not present in brain, which maintains ~30% residual CoQ and is clinically unaffected. In Pdss2kd/kd mice, we also observed low levels of plasma and urine thiosulfate and increased blood C4‐C6 acylcarnitines. We propose that impairment of the sulfide oxidation pathway induced by decreased levels of CoQ causes accumulation of sulfides and consequent inhibition of short‐chain acyl‐CoA dehydrogenase and glutathione depletion, which contributes to increased oxidative stress and kidney failure.
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title_short	Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway
url	https://doi.org/10.15252/emmm.201606356 https://doaj.org/article/c93d874d27cf40a7a84b8c58b34251f8 https://doaj.org/toc/1757-4676 https://doaj.org/toc/1757-4684
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up_date	2024-07-03T17:29:52.550Z
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Here, we show that lack of CoQ in human skin fibroblasts causes impairment of hydrogen sulfide oxidation, proportional to the residual levels of CoQ. Biochemical and molecular abnormalities are rescued by CoQ supplementation in vitro and recapitulated by pharmacological inhibition of CoQ biosynthesis in skin fibroblasts and ADCK3 depletion in HeLa cells. Kidneys of Pdss2kd/kd mice, which only have ~15% residual CoQ concentrations and are clinically affected, showed (i) reduced protein levels of SQR and downstream enzymes, (ii) accumulation of hydrogen sulfides, and (iii) glutathione depletion. These abnormalities were not present in brain, which maintains ~30% residual CoQ and is clinically unaffected. In Pdss2kd/kd mice, we also observed low levels of plasma and urine thiosulfate and increased blood C4‐C6 acylcarnitines. We propose that impairment of the sulfide oxidation pathway induced by decreased levels of CoQ causes accumulation of sulfides and consequent inhibition of short‐chain acyl‐CoA dehydrogenase and glutathione depletion, which contributes to increased oxidative stress and kidney failure.</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">coenzyme Q</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">CoQ10</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Pdss2</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">SQR</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">sulfides</subfield></datafield><datafield tag="653" ind1=" " ind2="0"><subfield code="a">Medicine (General)</subfield></datafield><datafield tag="653" ind1=" " ind2="0"><subfield code="a">Genetics</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">Ivano Di 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Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway

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