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MetabolomicsSequential lipidomic, metabolomic, and proteomic analyses of serum, liver, and heart tissue specimens from peroxisomal biogenesis factor 11α knockout mice
Peroxisomes are versatile single membrane-enclosed cytoplasmic organelles, involved in reactive oxygen species (ROS) and lipid metabolism and diverse other metabolic processes. Peroxisomal disorders result from mutations in Pex genes-encoded proteins named peroxins (PEX proteins) and single peroxiso...
2022-02-02 | MTBLS1887 | MetaboLights
MetabolomicsInfection-Induced Peroxisome Biogenesis Is a Metabolic Strategy for Herpesvirus Replication
Peroxisomes are primarily metabolic organelles with important functions in lipid metabolism, such as fatty acid oxidation and ether phospholipid synthesis (e.g. plasmalogens). Certain viruses, such as human cytomegalovirus (HCMV), hijack organelle functions to facilitate their replication and spread...
2018-10-22 | MTBLS652 | MetaboLights
TranscriptomicsA Drosophila model for the Zellweger spectrum of peroxisome biogenesis disorders
Human peroxisome biogenesis disorders are lethal genetic disease in which abnormal peroxisome assembly compromises overall peroxisome and cellular function. Peroxisomes are ubiquitous membrane-bound organelles involved in several important biochemical processes, notably lipid metabolism and the use ...
ORGANISM(S): Drosophila melanogaster 
2011-07-02 | E-GEOD-30362 | biostudies-arrayexpress
ProteomicsThe biochemical basis of mitochondrial dysfunctionin Zellweger Spectrum Disorder
Peroxisomal Biogenesis Disorders (PBDs) are genetic disorders of peroxisome biogenesis and metabolism that are characterized by profound developmental and neurological phenotypes. The most severe class of PBDs—Zellweger Spectrum Disorder(ZSD)—is caused by mutations in peroxin genes that result in b...
ORGANISM(S): Saccharomyces cerevisiae (Baker's yeast) 
2021-05-10 | PXD025595 | Pride
Zellweger spectrum disorder Mitochondria Peroxisomal biogenesis disorders Lc-ms/ms 
ProteomicsThe biochemical basis of mitochondrial dysfunction in Zellweger Spectrum Disorder
We identified that peroxins were still expressed in Zellweger Spectrum Disorder (ZSD) and a subset of them accumulated on the mitochondrial membrane, which resulted in gross mitochondrial abnormalities and impaired mitochondrial metabolic function. In this complexome analysis we detected several per...
ORGANISM(S): Saccharomyces cerevisiae (Baker's yeast) 
2021-06-25 | PXD024625 | Pride
Peroxisomes Mitocondrial quality control Mitochondria Peroxisomal biogenesis disorder Peroxisomal import Lc-msms Complexome profiling 
UnknownThe TRIM37 gene encodes a peroxisomal RING-B-box-coiled-coil protein: classification of mulibrey nanism as a new peroxisomal disorder.
Not available
S-EPMC447596 | biostudies-literature
UnknownA PEX6-defective peroxisomal biogenesis disorder with severe phenotype in an infant, versus mild phenotype resembling Usher syndrome in the affected parents.
Not available
S-EPMC379104 | biostudies-literature
TranscriptomicsDNA methylation profiles of fibroblasts and induced pluripotent stem cells (iPSCs) from individuals with Zellweger spectrum disorder (ZSD), a class of peroxisome biogenesis disorder, and healthy controls
Zellweger spectrum disorder (PBD-ZSD) is a disease continuum caused by mutations in a subset of PEX genes required for normal peroxisome assembly and function. Their clinical manifestations highlight the importance of peroxisomes in the development and functions of the central nervous system, liver,...
ORGANISM(S): Homo sapiens 
2015-05-19 | E-GEOD-68134 | biostudies-arrayexpress
TranscriptomicsThe biochemical basis of mitochondrial dysfunction in Zellweger Spectrum Disorder 
Peroxisomal Biogenesis Disorders (PBDs) are genetic disorders of peroxisome biogenesis and metabolism that are characterized by profound developmental and neurological phenotypes. The most severe class of PBDs—Zellweger Spectrum Disorder (ZSD)—is caused by mutations in peroxin genes that result in b...
ORGANISM(S): Saccharomyces cerevisiae 
2021-06-13 | GSE159869 | GEO
UnknownArginine improves peroxisome functioning in cells from patients with a mild peroxisome biogenesis disorder.
Not available
S-EPMC3844471 | biostudies-literature
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