cDNA Cloning of the Human Peroxisomal Enoyl-CoA Hydratase

3-Hydroxyacyl-CoA Dehydrogenase Bifunctional Enzyme and Localization to Chromosome 3q26.3-3q28: A Free Left Alu Arm Is Inserted in the 3′ Noncoding Region

Gerald Hoefler, Michael Forstner, Martina C. McGuinness, Wolfgang Hulla, Michaela Hiden, Peter Krisper, Lukas Kenner, Thomas Ried, Christoph Lengauer, Rudolf Zechner, Hugo W. Moser, Grace L. Chen

Research output: Contribution to journalArticle

Abstract

Enoyl-CoA hydratase:3-hydroxyacyl-CoA dehydrogenase bifunctional enzyme is one of the four enzymes of the peroxisomal β-oxidation pathway. Here, we report the full-length human cDNA sequence and the localization of the corresponding gene on chromosome 3q26.3-3q28. The cDNA sequence spans 3779 nucleotides with an open reading frame of 2169 nucleotides. The tripeptide SKL at the carboxy terminus, known to serve as a peroxisomal targeting signal, is present. DNA sequence comparison of the coding region showed an 80% homology between human and rat bifunctional enzyme cDNA. The 3′ noncoding sequence contains 117 nucleotides homologous to an Alu repeat. Based on sequence comparison, we propose that these nucleotides are a free left Alu arm with 86% homology to the Alu-J family. RNA analysis shows one band with highest intensity in liver and kidney. This cDNA will allow in-depth studies of molecular defects in patients with defective peroxisomal bifunctional enzyme. Moreover, it will also provide a means for studying the regulation of peroxisomal β-oxidation in humans.

Original languageEnglish (US)
Pages (from-to)60-67
Number of pages8
JournalGenomics
Volume19
Issue number1
DOIs
StatePublished - Jan 1 1994
Externally publishedYes

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Enoyl-CoA Hydratase
3-Hydroxyacyl-CoA Dehydrogenase
Organism Cloning
Nucleotides
Complementary DNA
Chromosomes
Enzymes
seryl-lysyl-leucine
Peroxisomal Bifunctional Enzyme
Alu Elements
Open Reading Frames
RNA
Kidney
Liver
Genes

ASJC Scopus subject areas

  • Genetics

Cite this

cDNA Cloning of the Human Peroxisomal Enoyl-CoA Hydratase : 3-Hydroxyacyl-CoA Dehydrogenase Bifunctional Enzyme and Localization to Chromosome 3q26.3-3q28: A Free Left Alu Arm Is Inserted in the 3′ Noncoding Region. / Hoefler, Gerald; Forstner, Michael; McGuinness, Martina C.; Hulla, Wolfgang; Hiden, Michaela; Krisper, Peter; Kenner, Lukas; Ried, Thomas; Lengauer, Christoph; Zechner, Rudolf; Moser, Hugo W.; Chen, Grace L.

In: Genomics, Vol. 19, No. 1, 01.01.1994, p. 60-67.

Research output: Contribution to journalArticle

Hoefler, G, Forstner, M, McGuinness, MC, Hulla, W, Hiden, M, Krisper, P, Kenner, L, Ried, T, Lengauer, C, Zechner, R, Moser, HW & Chen, GL 1994, 'cDNA Cloning of the Human Peroxisomal Enoyl-CoA Hydratase: 3-Hydroxyacyl-CoA Dehydrogenase Bifunctional Enzyme and Localization to Chromosome 3q26.3-3q28: A Free Left Alu Arm Is Inserted in the 3′ Noncoding Region', Genomics, vol. 19, no. 1, pp. 60-67. https://doi.org/10.1006/geno.1994.1013
Hoefler, Gerald ; Forstner, Michael ; McGuinness, Martina C. ; Hulla, Wolfgang ; Hiden, Michaela ; Krisper, Peter ; Kenner, Lukas ; Ried, Thomas ; Lengauer, Christoph ; Zechner, Rudolf ; Moser, Hugo W. ; Chen, Grace L. / cDNA Cloning of the Human Peroxisomal Enoyl-CoA Hydratase : 3-Hydroxyacyl-CoA Dehydrogenase Bifunctional Enzyme and Localization to Chromosome 3q26.3-3q28: A Free Left Alu Arm Is Inserted in the 3′ Noncoding Region. In: Genomics. 1994 ; Vol. 19, No. 1. pp. 60-67.
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