Peroxisomal Bifunctional Enzyme

Known as: Peroxisomal Multifunctional beta-Oxidation Protein, L Bifunctional Enzyme, Peroxisomal Hydratase Dehydrogenase Epimerase 
A monomeric protein found in liver peroxisomes that contains two enzymatically active domains; an enoyl-CoA hydratase/3,2-trans-enoyl-CoA isomerase… (More)
National Institutes of Health

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EHHADH geneIn BloodProcess of secretionantagonists & inhibitors
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EHHADH protein, human

Papers overview

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Highly Cited
2004
Highly Cited
2004
Comparative in vivo stability of copper-64-labeled cross-bridged and conventional tetraazamacrocyclic complexes.
The increased use of copper radioisotopes in radiopharmaceutical applications has created a need for bifunctional chelators (BFCs… (More)
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Highly Cited
2003
Highly Cited
2003
89Zr immuno-PET: comprehensive procedures for the production of 89Zr-labeled monoclonal antibodies.
UNLABELLED The use of immuno-PET, the combination of PET with monoclonal antibodies (mAbs), is an attractive option to improve… (More)
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Highly Cited
2002
Highly Cited
2002
Gemtuzumab ozogamicin, a potent and selective anti-CD33 antibody-calicheamicin conjugate for treatment of acute myeloid leukemia.
CD33 is expressed by acute myeloid leukemia (AML) cells in >80% of patients but not by normal hematopoietic stem cells… (More)
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Highly Cited
2000
Highly Cited
2000
Peroxisomal targeting signal-1 recognition by the TPR domains of human PEX5
Many proteins contain targeting signals within their sequences that specify their delivery to particular organelles. The… (More)
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1999
1999
Decreased expression of the peroxisomal bifunctional enzyme and carbonyl reductase in human hepatocellular carcinomas
Human hepatocellular carcinomas (HCC) are known to frequently exhibit clear-cell or fatty change. The expression of three enzymes… (More)
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1997
1997
Peroxisomal bifunctional enzyme deficiency with associated retinal findings.
Peroxisomal disorders include single enzyme defects and defects of peroxisomal fatty acid oxidation enzymes. Peroxisomal… (More)
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1996
1996
Neuronal migration abnormality in peroxisomal bifunctional enzyme defect.
Patterns of brain dysgenesis that resemble those in the Zellweger syndrome were demonstrated in a boy with an isolated defect of… (More)
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1996
1996
Urinary bile acids and peroxisomal bifunctional enzyme deficiency.
The biosynthesis of normal bile acids involves beta-oxidation of the 8-carbon side-chain of cholesterol, in addition to numerous… (More)
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1995
1995
Distinction between peroxisomal bifunctional enzyme and acyl-CoA oxidase deficiencies.
The clinical distinction between patients with a disorder of peroxisome assembly (e.g., Zellweger syndrome) and those with a… (More)
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Highly Cited
1989
Highly Cited
1989
Peroxisomal bifunctional enzyme deficiency.
Peroxisomal function was evaluated in a male infant with clinical features of neonatal adrenoleukodystrophy. Very long chain… (More)
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