'Chemical co-substrate rescue' of phytanoyl-CoA 2-hydroxylase mutants causing Refsum's Disease

Identification of the cytochrome P450 enzymes responsible for the omega-hydroxylation of phytanic acid

Komen, J. C.
Wanders, R. J. A.

January 2006

Patients suffering from Refsum disease have a defect in the alpha-oxidation pathway which results in...

Restoration of phytanic acid oxidation in Refsum disease fibroblasts from patients with mutations in the phytanoyl-CoA hydroxylase gene

Chahal, Amarjit
Khan, Mushfiquddin
Pai, Shashidhar G
Barbosa, Ernest
Singh, Inderjit

June 1998

AbstractRefsum disease (RD) is biochemically characterized by the excessive accumulation of phytanic...

Omega-hydroxylation of phytanic acid in rat liver microsomes: implications for Refsum disease

Komen, J. C.
Duran, M.
Wanders, R. J. A.

January 2004

The 3-methyl-branched fatty acid phytanic acid is degraded by the peroxisomal alpha-oxidation route ...

'Chemical co-substrate rescue' of phytanoyl-CoA 2-hydroxylase mutants causing Refsum's Disease

Mukherji, M
Kershaw, NJ
MacKinnon, CH
Clifton, IJ
Wierzbicki, AS
Schofield, CJ
Lloyd, MD

January 2001

The in vitro catalytic activity of two clinically observed mutants of phytanoyl-CoA 2-hydroxylase, a...

Structure-function analysis of phytanoyl-CoA 2-hydroxylase mutations causing Refsum's disease.

Mukherji, M
Chien, W
Kershaw, NJ
Clifton, IJ
Schofield, CJ
Wierzbicki, AS
Lloyd, MD

September 2001

Refsum's disease is a neurological syndrome characterized by adult-onset retinitis pigmentosa, anosm...

Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease.

McDonough, M
Kavanagh, K
Butler, D
Searls, T
Oppermann, U
Schofield, C

January 2005

Refsum disease (RD), a neurological syndrome characterized by adult onset retinitis pigmentosa, anos...

Structure-function analysis of phytanoyl-CoA 2-hydroxylase mutations causing Refsum's disease

Mukherji, M
Chien, W
Kershaw, N J
Clifton, I J
Schofield, C J
Wierzbicki, A S
Lloyd, M D

January 2001

Refsum's disease is a neurological syndrome characterized by adult-onset retinitis pigmentosa, anosm...

Studies on the specificity of unprocessed and mature forms of phytanoyl-CoA 2-hydroxylase and mutation of the iron binding ligands.

Searls, T
Butler, D
Chien, W
Mukherji, M
Lloyd, MD
Schofield, CJ

August 2005

The mature form of phytanoyl-coenzyme A 2-hydroxylase (PAHX), a nonheme Fe(II)- and 2-oxoglutarate-d...

Structural and mechanistic studies on the peroxisomal oxygenase phytanoyl-CoA 2-hydroxylase (PhyH).

Schofield, C
McDonough, M

January 2007

Phytanic acid (PA) is an epimeric metabolite of the isoprenoid side chain of chlorophyll. Owing to t...

Utilization of sterol carrier protein-2 by phytanoyl-CoA 2-hydroxylase in the peroxisomal alpha oxidation of phytanic acid.

Mukherji, M
Kershaw, NJ
Schofield, CJ
Wierzbicki, AS
Lloyd, MD

January 2002

Since it possesses a 3-methyl group, phytanic acid is degraded by a peroxisomal alpha-oxidation path...

Utilization of Sterol Carrier Protein-2 by Phytanoyl-CoA 2-Hydroxylase in the Peroxisomal α Oxidation of Phytanic Acid

Mukherji, Mridul
Kershaw, Nadia J.
Schofield, Christopher J.
Wierzbicki, Anthony S.
Lloyd, Matthew D.

May 2002

AbstractSince it possesses a 3-methyl group, phytanic acid is degraded by a peroxisomal α-oxidation ...

Identification of the cytochrome P450 enzymes responsible for the ω-hydroxylation of phytanic acid

Komen, J.C.
Wanders, R.J.A.

July 2006

AbstractPatients suffering from Refsum disease have a defect in the α-oxidation pathway which result...

Alteration of the co-substrate selectivity of deacetoxycephalosporin C synthase. The role of arginine 258.

Lee, H
Lloyd, MD
Clifton, I
Harlos, K
Dubus, A
Baldwin, J
Frere, J
Schofield, C

January 2001

Deacetoxycephalosporin C synthase is an iron(II) 2-oxoglutaratedependent oxygenase that catalyzes th...

Modulation of the Activity of Newly Synthesized Human Phenylalanine Hydroxylase Mutant Proteins by Low-Molecular-Weight Compounds

Nascimento, Catia
Leandro, Joao
de Almeida, Isabel Tavares
Leandro, Paula

January 2008

Phenylketonuria, the most frequent disorder of amino acid metabolism, is caused by a deficient activ...

Studies on phytanoyl-CoA 2-hydroxylase and synthesis of phytanoyl-coenzyme A.

Kershaw, NJ
Mukherji, M
MacKinnon, CH
Claridge, TD
Odell, B
Wierzbicki, AS
Lloyd, MD
Schofield, CJ

September 2001

Phytanoyl-CoA 2-hydroxylase (PAHX), an iron(II) and 2-oxoglutarate-dependent oxygenase, catalyses an...

Identification of the cytochrome P450 enzymes responsible for the omega-hydroxylation of phytanic acid

Komen, J. C.
Wanders, R. J. A.

January 2006

Patients suffering from Refsum disease have a defect in the alpha-oxidation pathway which results in...

Restoration of phytanic acid oxidation in Refsum disease fibroblasts from patients with mutations in the phytanoyl-CoA hydroxylase gene

Chahal, Amarjit
Khan, Mushfiquddin
Pai, Shashidhar G
Barbosa, Ernest
Singh, Inderjit

June 1998

AbstractRefsum disease (RD) is biochemically characterized by the excessive accumulation of phytanic...

Omega-hydroxylation of phytanic acid in rat liver microsomes: implications for Refsum disease

Komen, J. C.
Duran, M.
Wanders, R. J. A.

January 2004

The 3-methyl-branched fatty acid phytanic acid is degraded by the peroxisomal alpha-oxidation route ...

'Chemical co-substrate rescue' of phytanoyl-CoA 2-hydroxylase mutants causing Refsum's Disease

Mukherji, M
Kershaw, NJ
MacKinnon, CH
Clifton, IJ
Wierzbicki, AS
Schofield, CJ
Lloyd, MD

January 2001

The in vitro catalytic activity of two clinically observed mutants of phytanoyl-CoA 2-hydroxylase, a...

Structure-function analysis of phytanoyl-CoA 2-hydroxylase mutations causing Refsum's disease.

Mukherji, M
Chien, W
Kershaw, NJ
Clifton, IJ
Schofield, CJ
Wierzbicki, AS
Lloyd, MD

September 2001

Refsum's disease is a neurological syndrome characterized by adult-onset retinitis pigmentosa, anosm...

Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease.

McDonough, M
Kavanagh, K
Butler, D
Searls, T
Oppermann, U
Schofield, C

January 2005

Refsum disease (RD), a neurological syndrome characterized by adult onset retinitis pigmentosa, anos...

Structure-function analysis of phytanoyl-CoA 2-hydroxylase mutations causing Refsum's disease

Mukherji, M
Chien, W
Kershaw, N J
Clifton, I J
Schofield, C J
Wierzbicki, A S
Lloyd, M D

January 2001

Refsum's disease is a neurological syndrome characterized by adult-onset retinitis pigmentosa, anosm...

Studies on the specificity of unprocessed and mature forms of phytanoyl-CoA 2-hydroxylase and mutation of the iron binding ligands.

Searls, T
Butler, D
Chien, W
Mukherji, M
Lloyd, MD
Schofield, CJ

August 2005

The mature form of phytanoyl-coenzyme A 2-hydroxylase (PAHX), a nonheme Fe(II)- and 2-oxoglutarate-d...

Structural and mechanistic studies on the peroxisomal oxygenase phytanoyl-CoA 2-hydroxylase (PhyH).

Schofield, C
McDonough, M

January 2007

Phytanic acid (PA) is an epimeric metabolite of the isoprenoid side chain of chlorophyll. Owing to t...

Utilization of sterol carrier protein-2 by phytanoyl-CoA 2-hydroxylase in the peroxisomal alpha oxidation of phytanic acid.

Mukherji, M
Kershaw, NJ
Schofield, CJ
Wierzbicki, AS
Lloyd, MD

January 2002

Since it possesses a 3-methyl group, phytanic acid is degraded by a peroxisomal alpha-oxidation path...

Utilization of Sterol Carrier Protein-2 by Phytanoyl-CoA 2-Hydroxylase in the Peroxisomal α Oxidation of Phytanic Acid

Mukherji, Mridul
Kershaw, Nadia J.
Schofield, Christopher J.
Wierzbicki, Anthony S.
Lloyd, Matthew D.

May 2002

AbstractSince it possesses a 3-methyl group, phytanic acid is degraded by a peroxisomal α-oxidation ...

Identification of the cytochrome P450 enzymes responsible for the ω-hydroxylation of phytanic acid

Komen, J.C.
Wanders, R.J.A.

July 2006

AbstractPatients suffering from Refsum disease have a defect in the α-oxidation pathway which result...

Alteration of the co-substrate selectivity of deacetoxycephalosporin C synthase. The role of arginine 258.

Lee, H
Lloyd, MD
Clifton, I
Harlos, K
Dubus, A
Baldwin, J
Frere, J
Schofield, C

January 2001

Deacetoxycephalosporin C synthase is an iron(II) 2-oxoglutaratedependent oxygenase that catalyzes th...

Modulation of the Activity of Newly Synthesized Human Phenylalanine Hydroxylase Mutant Proteins by Low-Molecular-Weight Compounds

Nascimento, Catia
Leandro, Joao
de Almeida, Isabel Tavares
Leandro, Paula

January 2008

Phenylketonuria, the most frequent disorder of amino acid metabolism, is caused by a deficient activ...

Studies on phytanoyl-CoA 2-hydroxylase and synthesis of phytanoyl-coenzyme A.

Kershaw, NJ
Mukherji, M
MacKinnon, CH
Claridge, TD
Odell, B
Wierzbicki, AS
Lloyd, MD
Schofield, CJ

September 2001

Phytanoyl-CoA 2-hydroxylase (PAHX), an iron(II) and 2-oxoglutarate-dependent oxygenase, catalyses an...

Identification of the cytochrome P450 enzymes responsible for the omega-hydroxylation of phytanic acid

Komen, J. C.
Wanders, R. J. A.

January 2006

Patients suffering from Refsum disease have a defect in the alpha-oxidation pathway which results in...

Restoration of phytanic acid oxidation in Refsum disease fibroblasts from patients with mutations in the phytanoyl-CoA hydroxylase gene

Chahal, Amarjit
Khan, Mushfiquddin
Pai, Shashidhar G
Barbosa, Ernest
Singh, Inderjit

June 1998

AbstractRefsum disease (RD) is biochemically characterized by the excessive accumulation of phytanic...

Omega-hydroxylation of phytanic acid in rat liver microsomes: implications for Refsum disease

Komen, J. C.
Duran, M.
Wanders, R. J. A.

January 2004

The 3-methyl-branched fatty acid phytanic acid is degraded by the peroxisomal alpha-oxidation route ...

'Chemical co-substrate rescue' of phytanoyl-CoA 2-hydroxylase mutants causing Refsum's Disease

Abstract

Extracted data

'Chemical co-substrate rescue' of phytanoyl-CoA 2-hydroxylase mutants causing Refsum's Disease

Abstract

Extracted data

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