Zobrazeno 1 - 10
of 137
pro vyhledávání: '"Refsum's disease"'
Autor:
Wedad Fallatah, Wei Cui, Erminia Di Pietro, Grace T. Carter, Brittany Pounder, Fabian Dorninger, Christian Pifl, Ann B. Moser, Johannes Berger, Nancy E. Braverman
Publikováno v:
Frontiers in Cell and Developmental Biology, Vol 10 (2022)
Rhizomelic chondrodysplasia punctata type 1 (RCDP1) is a peroxisome biogenesis disorder caused by defects in PEX7 leading to impairment in plasmalogen (Pls) biosynthesis and phytanic acid (PA) oxidation. Pls deficiency is the main pathogenic factor t
Externí odkaz:
https://doaj.org/article/77a6359dc970481a89c729b71f113d34
Akademický článek
Tento výsledek nelze pro nepřihlášené uživatele zobrazit.
K zobrazení výsledku je třeba se přihlásit.
K zobrazení výsledku je třeba se přihlásit.
Autor:
Timothy Searls, Danica Butler, Winnie Chien, Mridul Mukherji, Matthew D. Lloyd, Christopher J. Schofield
Publikováno v:
Journal of Lipid Research, Vol 46, Iss 8, Pp 1660-1667 (2005)
The mature form of phytanoyl-coenzyme A 2-hydroxylase (PAHX), a nonheme Fe(II)- and 2-oxoglutarate-dependent oxygenase, catalyzes the α-hydroxylation of phytanoyl-CoA within peroxisomes. Mutations in PAHX result in some forms of adult Refsum's disea
Externí odkaz:
https://doaj.org/article/60cef5c4d0704254935a7959317f6b02
Publikováno v:
Journal of Lipid Research, Vol 41, Iss 4, Pp 629-636 (2000)
Phytanoyl-CoA hydroxylase is a peroxisomal α-oxidation enzyme that catalyzes the 2-hydroxylation of 3-methyl-branched acyl-CoAs. A polyhistidine-tagged human phytanoyl-CoA hydroxylase was expressed in E. coli and subsequently purified as an active p
Externí odkaz:
https://doaj.org/article/d14d9501d43a4a058b7a34ee084b7bff
Autor:
Gerbert A. Jansen, Rob Ofman, Simone Denis, Sacha Ferdinandusse, Eveline M. Hogenhout, Cornelis Jakobs, Ronald J.A. Wanders
Publikováno v:
Journal of Lipid Research, Vol 40, Iss 12, Pp 2244-2254 (1999)
Phytanoyl-CoA hydroxylase (PhyH) catalyzes the conversion of phytanoyl-CoA to 2-hydroxyphytanoyl-CoA, which is the first step in the phytanic acid α-oxidation pathway. Recently, several studies have shown that in humans, phytanic acid α-oxidation i
Externí odkaz:
https://doaj.org/article/583b1259093349ef9959548daf43ef60
Autor:
Kathleen Croes, Minne Casteels, Martine Dieuaide-Noubhani, Guy P. Mannaerts, Paul P. Van Veldhoven
Publikováno v:
Journal of Lipid Research, Vol 40, Iss 4, Pp 601-609 (1999)
The stereochemistry of the α-oxidation of 3-methyl-branched fatty acids was studied in rat liver. R- and S-3-methylhexadecanoic acid were equally well α-oxidized in intact hepatocytes and homogenates. Subcellular fractionation studies showed that
Externí odkaz:
https://doaj.org/article/699d88aee25544a2b568bada5e043649
Autor:
Rehiana Ali, Andrew J Larner
Publikováno v:
The Journal of the Royal College of Physicians of Edinburgh, Vol 49, Iss 4, Pp 309-310 (2019)
Autor:
Timothy Searls, Matthew D. Lloyd, Mridul Mukherji, Danica Butler, Winnie Chien, Christopher J. Schofield
Publikováno v:
Journal of Lipid Research, Vol 46, Iss 8, Pp 1660-1667 (2005)
The mature form of phytanoyl-coenzyme A 2-hydroxylase (PAHX), a nonheme Fe(II)- and 2-oxoglutarate-dependent oxygenase, catalyzes the alpha-hydroxylation of phytanoyl-CoA within peroxisomes. Mutations in PAHX result in some forms of adult Refsum's di
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::7466a3f1ee20829b2cbdc27395548e6d
https://doi.org/10.1194/jlr.m500034-jlr200
https://doi.org/10.1194/jlr.m500034-jlr200
Publikováno v:
Journal of Lipid Research, Vol 41, Iss 4, Pp 629-636 (2000)
Phytanoyl-CoA hydroxylase is a peroxisomal a - oxidation enzyme that catalyzes the 2-hydroxylation of 3- methyl-branched acyl-CoAs. A polyhistidine-tagged human phytanoyl-CoA hydroxylase was expressed in E. coli and sub- sequently purified as an acti
Autor:
Ronald J.A. Wanders, Eveline M. Hogenhout, Cornelis Jakobs, Simone Denis, Gerbert A. Jansen, Sacha Ferdinandusse, Rob Ofman
Publikováno v:
Journal of Lipid Research, Vol 40, Iss 12, Pp 2244-2254 (1999)
Phytanoyl-CoA hydroxylase (PhyH) catalyzes the conversion of phytanoyl-CoA to 2-hydroxyphytanoyl-CoA, which is the first step in the phytanic acid a -oxidation path- way. Recently, several studies have shown that in humans, phytanic acid a -oxidation