Unnatural co-substrate-based rescue of enzyme activity in Refsum's disease resulting from mutations in R275 of phytanoyl-CoA hydroxylase.

Unnatural co-substrate-based rescue of enzyme activity in Refsum's disease resulting from mutations in R275 of phytanoyl-CoA hydroxylase.

Detalles Bibliográficos
Main Authors:	Lloyd, MD, Mukherji, M, Kershaw, N, MacKinnon, C, Schofield, C, Wierzbicki, A
Formato:	Journal article
Publicado:	2000

Títulos similares

'Chemical co-substrate rescue' of phytanoyl-CoA 2-hydroxylase mutants causing Refsum's Disease
por: Mukherji, M, et al.
Publicado: (2001)

Structure-function analysis of phytanoyl-CoA 2-hydroxylase mutations causing Refsum's disease.
por: Mukherji, M, et al.
Publicado: (2001)

Studies on phytanoyl-CoA 2-hydroxylase and synthesis of phytanoyl-coenzyme A.
por: Kershaw, N, et al.
Publicado: (2001)

Role of phytanoyl-CoA 2-hydroxylase in phytanic acid metabolism.
por: Lloyd, MD, et al.
Publicado: (2003)

Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease.
por: McDonough, M, et al.
Publicado: (2005)

Utilization of sterol carrier protein-2 by phytanoyl-CoA 2-hydroxylase in the peroxisomal alpha oxidation of phytanic acid.
por: Mukherji, M, et al.
Publicado: (2002)

Studies on the specificity of unprocessed and mature forms of phytanoyl-CoA 2-hydroxylase and mutation of the iron binding ligands.
por: Searls, T, et al.
Publicado: (2005)

Further studies on the substrate spectrum of phytanoyl-CoA hydroxylase
por: Veerle Foulon, et al.
Publicado: (2003-12-01)

Studies on the specificity of unprocessed and mature forms of phytanoyl-CoA 2-hydroxylase and mutation of the iron binding ligands
por: Timothy Searls, et al.
Publicado: (2005-08-01)

Structural and mechanistic studies on the peroxisomal oxygenase phytanoyl-CoA 2-hydroxylase (PhyH).
por: Schofield, C, et al.
Publicado: (2007)

Crystal structure of PHYHD1A, a 2OG oxygenase related to phytanoyl-CoA hydroxylase.
por: Zhang, Z, et al.
Publicado: (2011)

Phytanoyl-CoA hydroxylase: recognition of 3-methyl-branched acyl-CoAs and requirement for GTP or ATP and Mg2+ in addition to its known hydroxylation cofactors
por: Kathleen Croes, et al.
Publicado: (2000-04-01)

Phytanoyl-CoA hydroxylase from rat liver: protein purification and cDNA cloning with implications for the subcellular localization of phytanic acid α-oxidation
por: Gerbert A. Jansen, et al.
Publicado: (1999-12-01)

Refsum's disease: a peroxisomal disorder affecting phytanic acid alpha-oxidation.
por: Wierzbicki, A, et al.
Publicado: (2002)

Phytanic acid oxidation: topographical localization of phytanoyl-CoA ligase and transport of phytanic acid into human peroxisomes.
por: K Pahan, et al.
Publicado: (1995-05-01)

Infantile Refsum Disease
por: J Gordon Millichap
Publicado: (1988-03-01)

Genetic testing for Refsum disease
por: Abeshi Andi, et al.
Publicado: (2017-10-01)

Metabolism of phytanic acid and 3-methyl-adipic acid excretion in patients with adult Refsum disease
por: Anthony S. Wierzbicki, et al.
Publicado: (2003-08-01)

Studies on the link between HMG-CoA reductase and cholesterol 7 alpha-hydroxylase in rat liver.
por: I Björkhem, et al.
Publicado: (1988-02-01)

Conventional and advanced MR imaging in infantile Refsum disease
por: Mustafa Kılıç, et al.
Publicado: (2015-06-01)

“Unnatural, unnatural, unnatural, unnatural unnatural” . . . but real? The Toolbox Murders (Dennis Donnelly, 1978) and the Exploitation of True Story Adaptations
por: Wickham Clayton
Publicado: (2016-07-01)

Use of methylmalonyl‐CoA epimerase in enhancing crotonase stereoselectivity
por: Hamed, R, et al.
Publicado: (2015)

Dilated cardiomyopathy revealing Refsum disease: a case report
por: Salim Arous, et al.
Publicado: (2024-09-01)

Absorption of chlorophyll phytol in normal man and in patients with Refsum's disease
por: James H. Baxter
Publicado: (1968-09-01)

Unnatural Oceans
por: Jeremy B.C. Jackson, et al.
Publicado: (2001-12-01)

Loss of stearoyl-CoA desaturase 1 rescues cardiac function in obese leptin-deficient mice
por: Pawel Dobrzyn, et al.
Publicado: (2010-08-01)

Separate peroxisomal oxidases for fatty acyl-CoAs and trihydroxycoprostanoyl-CoA in human liver
por: M Casteels, et al.
Publicado: (1990-10-01)

Improved synthesis of 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA)
por: A.I. Louw, et al.
Publicado: (1969-11-01)

Stimulation of HMG-CoA reductase as a consequence of phenobarbital-induced primary stimulation of cholesterol 7 alpha-hydroxylase in rat liver.
por: E Sudjana-Sugiaman, et al.
Publicado: (1994-02-01)

Refsum disease: a defect in the alpha-oxidation of phytanic acid in peroxisomes
por: I Singh, et al.
Publicado: (1993-10-01)

Acetyl-CoA synthetase (ACSS2) does not generate butyryl- and crotonyl-CoA
por: Nour Zeaiter, et al.
Publicado: (2024-03-01)

Metabolism of acetyl-CoA by isolated peroxisomal fractions: formation of acetate and acetoacetyl-CoA.
por: R Hovik, et al.
Publicado: (1991-06-01)

Screening Phosphorylation Site Mutations in Yeast Acetyl-CoA Carboxylase Using Malonyl-CoA Sensor to Improve Malonyl-CoA-Derived Product
por: Xiaoxu Chen, et al.
Publicado: (2018-01-01)

Structural basis for expanded substrate specificities of human long chain acyl-CoA dehydrogenase and related acyl-CoA dehydrogenases
por: Beena Narayanan, et al.
Publicado: (2024-06-01)

Exploring the substrate selectivity of oxygen sensing prolyl hydroxylases
por: Lippl, K, et al.
Publicado: (2015)

Natural and unnatural silks
por: Holland, C, et al.
Publicado: (2007)

Natural and unnatural silks
por: Holland, C, et al.
Publicado: (2007)

Short- and long-term effects of ethanol administration in vivo on rat liver HMG-CoA reductase and cholesterol 7α-hydroxylase activities
por: M R Lakshmanan, et al.
Publicado: (1977-05-01)

Studies on the regulation of cholesterol 7 alpha-hydroxylase and HMG-CoA reductase in rat liver: effects of lymphatic drainage and ligation of the lymph duct.
por: JE Akerlund, et al.
Publicado: (1990-12-01)

Regulatory effects of sterols and bile acids on hepatic 3-hydroxy-3-methylglutaryl CoA reductase and cholesterol 7α-hydroxylase in the rat
por: S. Shefer, et al.
Publicado: (1973-09-01)