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Nudix Hydrolase 7 is an Acyl-CoA Diphosphatase Involved in Regulating Peroxisomal Coenzyme A Homeostasis

Journal of biochemistry (Tokyo), 2008-11, Vol.144 (5), p.655-663

2008

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Processing of mutant N-acetyl-α-glucosaminidase in mucopolysaccharidosis type IIIB fibroblasts cultured at low temperature

Molecular genetics and metabolism, 2017-09, Vol.122 (1-2), p.100-106

2017

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High-Throughput Screen Fails to Identify Compounds That Enhance Residual Enzyme Activity of Mutant N-Acetyl-α-Glucosaminidase in Mucopolysaccharidosis Type IIIB

JIMD Reports, Volume 39, 2018-01, Vol.39, p.97-106

2018

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Refsum disease is caused by mutations in the phytanoyl-CoA hydroxylase gene

Nature genetics, 1997-10, Vol.17 (2), p.190-193

1997

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Peroxisomal trans-2-enoyl-CoA reductase is involved in phytol degradation

FEBS letters, 2006-04, Vol.580 (8), p.2092-2096

2006

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Cholesterol-deprivation increases mono-unsaturated very long-chain fatty acids in skin fibroblasts from patients with X-linked adrenoleukodystrophy

Biochimica et biophysica acta, 2008-03, Vol.1781 (3), p.105-111

2008

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Acyl-CoA: Dihydroxyacetonephosphate Acyltransferase: Cloning of the Human cDNA and Resolution of the Molecular Basis in Rhizomelic Chondrodysplasia Punctata Type 2

Human molecular genetics, 1998-05, Vol.7 (5), p.847-853

1998

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Identification of Pristanal Dehydrogenase Activity in Peroxisomes: Conclusive Evidence That the Complete Phytanic Acid α-Oxidation Pathway Is Localized in Peroxisomes

Biochemical and biophysical research communications, 2001-05, Vol.283 (3), p.674-679

2001

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Demonstration of Dimethylnonanoyl-CoA Thioesterase Activity in Rat Liver Peroxisomes Followed by Purification and Molecular Cloning of the Thioesterase Involved

Biochemical and biophysical research communications, 2002-01, Vol.290 (2), p.629-634

2002

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Proteomic analysis of mouse kidney peroxisomes: identification of RP2p as a peroxisomal nudix hydrolase with acyl-CoA diphosphatase activity

Biochemical journal, 2006-01, Vol.393 (Pt 2), p.537-543

2006

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DO04 X-linked adrenoleukodystrophy: cholesterol-depletion increases mono-unsaturated very long chain fatty acids

European journal of paediatric neurology, 2007, Vol.11, p.39-39

2007

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Identification of fatty acid oxidation disorder patients with lowered acyl-CoA thioesterase activity in human skin fibroblasts

European journal of clinical investigation, 2005-01, Vol.35 (1), p.38-46

2005

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Omega-oxidation of very long-chain fatty acids in human liver microsomes. Implications for X-linked adrenoleukodystrophy

The Journal of biological chemistry, 2006-05, Vol.281 (19), p.13180-13187

2006

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2-Methyl-3-Hydroxybutyryl-CoA Dehydrogenase Deficiency Is Caused by Mutations in the HADH2 Gene

American journal of human genetics, 2003-05, Vol.72 (5), p.1300-1307

2003

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Elongation of very long-chain fatty acids is enhanced in X-linked adrenoleukodystrophy

Molecular genetics and metabolism, 2005-02, Vol.84 (2), p.144-151

2005

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Evidence for two enzymatic pathways for ω-oxidation of docosanoic acid in rat liver microsomes

Journal of lipid research, 2005-05, Vol.46 (5), p.1001-1008

2005

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2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency : An X-linked inborn error of isoleucine metabolism that may mimic a mitochondrial disease

Pediatric research, 2005-09, Vol.58 (3), p.488-491

2005

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Molecular and Biochemical Characterization of Rat ε-N-Trimethyllysine Hydroxylase, the First Enzyme of Carnitine Biosynthesis

The Journal of biological chemistry, 2001-09, Vol.276 (36), p.33512-33517

2001

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Immunoblot analysis of peroxisomal proteins in liver and fibroblasts from patients

Journal of inherited metabolic disease, 1995-01, Vol.18 (S1), p.101-112

1995

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Molecular and Biochemical Characterization of Rat γ-Trimethylaminobutyraldehyde Dehydrogenase and Evidence for the Involvement of Human Aldehyde Dehydrogenase 9 in Carnitine Biosynthesis

The Journal of biological chemistry, 2000-03, Vol.275 (10), p.7390-7394

2000

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Human phytanoyl-CoA hydroxylase : resolution of the gene structure and the molecular basis of Refsum's disease

Human molecular genetics, 2000-05, Vol.9 (8), p.1195-1200

2000

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Clinical, biochemical, and molecular findings in three patients with 3-hydroxyisobutyric aciduria

Molecular genetics and metabolism, 2006-03, Vol.87 (3), p.243-248

2006

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Rhizomelic chondrodysplasia punctata : deficiency of 3-oxoacyl-coenzyme A thiolase in peroxisomes and impaired processing of the enzyme

The Journal of clinical investigation, 1990-07, Vol.86 (1), p.126-130

1990

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Spastic diplegia and periventricular white matter abnormalities in 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency, a defect of isoleucine metabolism: differential diagnosis with hypoxic–ischemic brain diseases

Molecular genetics and metabolism, 2004-04, Vol.81 (4), p.295-299

2004

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Etherphospholipid Biosynthesis and Dihydroxyactetone-Phosphate Acyltransferase: Resolution of the Genomic Organization of the Human GNPAT Gene and Its Use in the Identification of Novel Mutations

Biochemical and biophysical research communications, 2001-03, Vol.281 (3), p.754-760

2001

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Nudix Hydrolase 7 is an Acyl-CoA Diphosphatase Involved in Regulating Peroxisomal Coenzyme A Homeostasis

Processing of mutant N-acetyl-α-glucosaminidase in mucopolysaccharidosis type IIIB fibroblasts cultured at low temperature

High-Throughput Screen Fails to Identify Compounds That Enhance Residual Enzyme Activity of Mutant N-Acetyl-α-Glucosaminidase in Mucopolysaccharidosis Type IIIB

Refsum disease is caused by mutations in the phytanoyl-CoA hydroxylase gene

Peroxisomal trans-2-enoyl-CoA reductase is involved in phytol degradation

Cholesterol-deprivation increases mono-unsaturated very long-chain fatty acids in skin fibroblasts from patients with X-linked adrenoleukodystrophy

Acyl-CoA: Dihydroxyacetonephosphate Acyltransferase: Cloning of the Human cDNA and Resolution of the Molecular Basis in Rhizomelic Chondrodysplasia Punctata Type 2

Identification of Pristanal Dehydrogenase Activity in Peroxisomes: Conclusive Evidence That the Complete Phytanic Acid α-Oxidation Pathway Is Localized in Peroxisomes

Demonstration of Dimethylnonanoyl-CoA Thioesterase Activity in Rat Liver Peroxisomes Followed by Purification and Molecular Cloning of the Thioesterase Involved

Proteomic analysis of mouse kidney peroxisomes: identification of RP2p as a peroxisomal nudix hydrolase with acyl-CoA diphosphatase activity

DO04 X-linked adrenoleukodystrophy: cholesterol-depletion increases mono-unsaturated very long chain fatty acids

Identification of fatty acid oxidation disorder patients with lowered acyl-CoA thioesterase activity in human skin fibroblasts

Omega-oxidation of very long-chain fatty acids in human liver microsomes. Implications for X-linked adrenoleukodystrophy

2-Methyl-3-Hydroxybutyryl-CoA Dehydrogenase Deficiency Is Caused by Mutations in the HADH2 Gene

Elongation of very long-chain fatty acids is enhanced in X-linked adrenoleukodystrophy

Evidence for two enzymatic pathways for ω-oxidation of docosanoic acid in rat liver microsomes

2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency : An X-linked inborn error of isoleucine metabolism that may mimic a mitochondrial disease

Molecular and Biochemical Characterization of Rat ε-N-Trimethyllysine Hydroxylase, the First Enzyme of Carnitine Biosynthesis

Immunoblot analysis of peroxisomal proteins in liver and fibroblasts from patients

Molecular and Biochemical Characterization of Rat γ-Trimethylaminobutyraldehyde Dehydrogenase and Evidence for the Involvement of Human Aldehyde Dehydrogenase 9 in Carnitine Biosynthesis

Human phytanoyl-CoA hydroxylase : resolution of the gene structure and the molecular basis of Refsum's disease

Clinical, biochemical, and molecular findings in three patients with 3-hydroxyisobutyric aciduria

Rhizomelic chondrodysplasia punctata : deficiency of 3-oxoacyl-coenzyme A thiolase in peroxisomes and impaired processing of the enzyme

Spastic diplegia and periventricular white matter abnormalities in 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency, a defect of isoleucine metabolism: differential diagnosis with hypoxic–ischemic brain diseases

Etherphospholipid Biosynthesis and Dihydroxyactetone-Phosphate Acyltransferase: Resolution of the Genomic Organization of the Human GNPAT Gene and Its Use in the Identification of Novel Mutations

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