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A lethal defect of mitochondrial and peroxisomal fission.
We report on a newborn girl with microcephaly, abnormal brain development, optic atrophy and hypoplasia, persistent lactic acidemia, and a mildly elevated plasma concentration of very-long-chainExpand
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Analysis of the Carboxyl-terminal Peroxisomal Targeting Signal 1 in a Homologous Context in Saccharomyces cerevisiae*
Most peroxisomal matrix proteins contain a carboxyl-terminal tripeptide that directs them to peroxisomes. Within limits, these amino acids may be varied, without loss of function. The specificity ofExpand
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Molecular characterization of carnitine‐dependent transport of acetyl‐CoA from peroxisomes to mitochondria in Saccharomyces cerevisiae and identification of a plasma membrane carnitine transporter,
In Saccharomyces cerevisiae, β‐oxidation of fatty acids is confined to peroxisomes. The acetyl‐CoA produced has to be transported from the peroxisomes via the cytoplasm to the mitochondrial matrix inExpand
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The role of ELOVL1 in very long-chain fatty acid homeostasis and X-linked adrenoleukodystrophy
X‐linked adrenoleukodystrophy (X‐ALD) is caused by mutations in the ABCD1 gene encoding the peroxisomal ABC transporter adrenoleukodystrophy protein (ALDP). X‐ALD is characterized by the accumulationExpand
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Peroxisomal Fatty Acid β-Oxidation Is Not Essential for Virulence of Candida albicans
ABSTRACT Phagocytic cells form the first line of defense against infections by the human fungal pathogen Candida albicans. Recent in vitro gene expression data suggest that upon phagocytosis byExpand
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Metabolite transport across the peroxisomal membrane.
In recent years, much progress has been made with respect to the unravelling of the functions of peroxisomes in metabolism, and it is now well established that peroxisomes are indispensableExpand
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Systematic mapping of contact sites reveals tethers and a function for the peroxisome-mitochondria contact
The understanding that organelles are not floating in the cytosol, but rather held in an organized yet dynamic interplay through membrane contact sites, is altering the way we grasp cell biologicalExpand
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Peroxisomal fatty acid alpha- and beta-oxidation in humans: enzymology, peroxisomal metabolite transporters and peroxisomal diseases.
Peroxisomes are subcellular organelles with an indispensable role in cellular metabolism. The importance of peroxisomes for humans is stressed by the existence of a group of genetic diseases inExpand
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Intrinsic acyl-CoA thioesterase activity of a peroxisomal ATP binding cassette transporter is required for transport and metabolism of fatty acids
Peroxisomes are organelles that perform diverse metabolic functions in different organisms, but a common function is β-oxidation of a variety of long chain aliphatic, branched, and aromaticExpand
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Differential substrate specificities of human ABCD1 and ABCD2 in peroxisomal fatty acid β-oxidation.
The gene mutated in X-linked adrenoleukodystrophy (X-ALD) codes for the HsABCD1 protein, also named ALDP, which is a member of the superfamily of ATP-binding cassette (ABC) transporters and requiredExpand
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