B. Distel

Publications53
h-index23
Citations2,624
Highly Influential Citations155
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Saccharomyces cerevisiae Pex3p and Pex19p are required for proper localization and stability of peroxisomal membrane proteins
The mechanisms by which peroxisomal membrane proteins (PMPs) are targeted to and inserted into membranes are unknown, as are the required components. We show that among a collection of 16… Expand
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The ABC transporter proteins Pat1 and Pat2 are required for import of long‐chain fatty acids into peroxisomes of Saccharomyces cerevisiae.
Peroxisomes of Saccharomyces cerevisiae are the exclusive site of fatty acid beta‐oxidation. We have found that fatty acids reach the peroxisomal matrix via two independent pathways. The subcellular… Expand
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A unified nomenclature for peroxisome biogenesis factors
Ben Distel,* Ralf Erdmann, ¢ Stephen J. Gould, ~ Gtinter Blobel,I Denis I. Crane, I James M. Cregg,** Gabriele Dodt,* Yukio Fujiki, *~ Joel M. Goodman, ~ Wilhelm W. Just, D Jan A.K.W. Kiel, 11… Expand
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Rhizomelic chondrodysplasia punctata is a peroxisomal protein targeting disease caused by a non-functional PTS2 receptor
Rhizomelic chondrodysplasia punctata (RCDP) is an autosomal recessive disease characterized clinically by a disproportionately short stature primarily affecting the proximal parts of the extremities,… Expand
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Import of proteins into peroxisomes.
Peroxisomes are organelles that confine an important set of enzymes within their single membrane boundaries. In man, a wide variety of genetic disorders is caused by loss of peroxisome function. In… Expand
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Sequence of the PAS8 gene, the product of which is essential for biogenesis of peroxisomes in Saccharomyces cerevisiae.
In a genetic screen for mutants disturbed in peroxisomal functions we found that the laboratory 'wild type' strain YP102 behaved like a typical peroxisome assembly mutant. Here, we report the… Expand
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Ube3a reinstatement identifies distinct developmental windows in a murine Angelman syndrome model.
Angelman syndrome (AS) is a severe neurodevelopmental disorder that results from loss of function of the maternal ubiquitin protein ligase E3A (UBE3A) allele. Due to neuron-specific imprinting, the… Expand
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De Novo Mutations in Protein Kinase Genes CAMK2A and CAMK2B Cause Intellectual Disability.
Calcium/calmodulin-dependent protein kinase II (CAMK2) is one of the first proteins shown to be essential for normal learning and synaptic plasticity in mice, but its requirement for human brain… Expand
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Differential protein import deficiencies in human peroxisome assembly disorders
Two peroxisome targeting signals (PTSs) for matrix proteins have been well defined to date. PTS1 comprises a COOH-terminal tripeptide, SKL, and has been found in several matrix proteins, whereas PTS2… Expand
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Characterization of a transcriptional control element involved in proliferation of peroxisomes in yeast in response to oleate.
Oleate induces the transcription of genes involved in peroxisome biogenesis and stimulates the proliferation of these organelles in Saccharomyces cerevisiae. Previously, we have reported the… Expand
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