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Clinical and biochemical characterization of four patients with mutations in ECHS1

Autor: Gallagher, Renata, Ferdinandusse, S, Friederich, MW, Burlina, A, Ruiter, JPN, Coughlin, CR, Dishop, MK, Gallagher, RC, Bedoyan, JK, Vaz, FM, Waterham, HR

Publikováno v: Gallagher, Renata; Ferdinandusse, S; Friederich, MW; Burlina, A; Ruiter, JPN; Coughlin, CR; et al.(2015). Clinical and biochemical characterization of four patients with mutations in ECHS1.. UC San Francisco: Retrieved from: http://www.escholarship.org/uc/item/1sv4j5jf

Short-chain enoyl-CoA hydratase (SCEH, encoded by ECHS1) catalyzes hydration of 2-trans-enoyl-CoAs to 3(S)-hydroxy-acyl-CoAs. SCEH has a broad substrate specificity and is believed to play an important role in mitochondrial fatty acid oxidation and i

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=od_______325::8b39a73d8af7feac636175cbfbdc90f9
http://www.escholarship.org/uc/item/1sv4j5jf

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cleavage of 3-oxovalproyl-CoA by a mitochondrial 3-oxoacyl-CoA thiolase

Autor: Silva, MFB, Ruiter, JPN, Overmars, H, Bootsma, AH, van Gennip, AH, Jakobs, C, Duran, M, de Alemida, IT, Wanders, RJA

The beta-oxidation of valproic acid (VPA; 2-n-propylpentanoic acid) was investigated in vitro in intact rat liver mitochondria incubated with H-3-labelled VPA. The metabolism of [4,5-H-3(2)]VPA and [2-H-3]VPA was studied by analysing the different ac

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=od______1961::0577985b68756cb9bc98b0d94043ce09
https://hdl.handle.net/10451/21333

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Complete beta-oxidation of valproate

Autor: Silva, MFB, Ruiter, JPN, Overmars, H, Bootsma, AH, van Gennip, AH, Jakobs, C, Duran, M, de Alemida, IT, Wanders, RJA

Publikováno v: Repositório Científico de Acesso Aberto de Portugal
Repositório Científico de Acesso Aberto de Portugal (RCAAP)
instacron:RCAAP

Made available in DSpace on 2015-12-30T10:17:57Z (GMT). No. of bitstreams: 0 Previous issue date: 2002

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=od______3056::0577985b68756cb9bc98b0d94043ce09

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Synthesis and intramitochondrial levels of valproyl-coenzyme A metabolites

Autor: Silva, MFB, Ruiter, JPN, IJlst, L, Allers, P, ten Brink, HJ, Jakobs, C, Duran, M, de Almedia, IT, Wander, RJA

Publikováno v: Repositório Científico de Acesso Aberto de Portugal
Repositório Científico de Acesso Aberto de Portugal (RCAAP)
instacron:RCAAP

A number of valproate adverse reactions are due to its interference with several metabolic pathways, including that of fatty acid oxidation. In order to resolve which mitochondrial enzymes of fatty acid oxidation are inhibited by which VPA intermedia

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=dedup_wf_001::bb91a91a154933f8312c807e2a0ee400

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Differential effect of valproate and its Delta(2)- and Delta(4)-unsaturated metabolites, on the beta-oxidation rate of long-chain and medium-chain fatty acids

Autor: Silva, MFB, Ruiter, JPN, IJlst, L, Jakobs, C, Duran, M, de Almeida, IT, Wanders, RJA

Publikováno v: Repositório Científico de Acesso Aberto de Portugal
Repositório Científico de Acesso Aberto de Portugal (RCAAP)
instacron:RCAAP

Overall fatty acid oxidation rates were investigated in rat hepatocytes using [9,10-H-3]palmitic, [9,10-H-3]-oleic, [9.10-H-3]-myristic and [2,3-H-3]-phenylpropionic acids. The effect of both valproate (VPA) (0-10 mM) and two of its unsaturated metab

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=dedup_wf_001::28b6c1771b0bdbe64192206e780a0514
https://hdl.handle.net/10451/21331

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