The physiological functions of human peroxisomes

RJA Wanders, M Baes, D Ribeiro… - Physiological …, 2023 - journals.physiology.org
Peroxisomes are subcellular organelles that play a central role in human physiology by
catalyzing a range of unique metabolic functions. The importance of peroxisomes for human …

Molecular insights into peroxisome homeostasis and peroxisome biogenesis disorders

Y Fujiki, K Okumoto, M Honsho, Y Abe - Biochimica et Biophysica Acta …, 2022 - Elsevier
Peroxisomes are single-membrane organelles essential for cell metabolism including the β-
oxidation of fatty acids, synthesis of etherlipid plasmalogens, and redox homeostasis …

PEX5 translocation into and out of peroxisomes drives matrix protein import

ML Skowyra, TA Rapoport - Molecular cell, 2022 - cell.com
Peroxisomes are ubiquitous organelles whose dysfunction causes fatal human diseases.
Most peroxisomal enzymes are imported from the cytosol by the receptor PEX5, which …

Shuttling mechanism of peroxisome targeting signal type 1 receptor Pex5: ATP-independent import and ATP-dependent export

N Miyata, Y Fujiki - Molecular and cellular biology, 2005 - Taylor & Francis
Peroxisomal matrix proteins are posttranslationally imported into peroxisomes with the
peroxisome-targeting signal 1 receptor, Pex5. The longer isoform of Pex5, Pex5L, also …

Towards solving the mystery of peroxisomal matrix protein import

ML Skowyra, P Feng, TA Rapoport - Trends in Cell Biology, 2024 - cell.com
Peroxisomes are vital metabolic organelles that import their lumenal (matrix) enzymes from
the cytosol using mobile receptors. Surprisingly, the receptors can even import folded …

Peroxisome biogenesis in mammalian cells

Y Fujiki, K Okumoto, S Mukai, M Honsho… - Frontiers in …, 2014 - frontiersin.org
To investigate peroxisome assembly and human peroxisome biogenesis disorders (PBDs)
such as Zellweger syndrome, thirteen different complementation groups (CGs) of Chinese …

Peroxisome biogenesis disorders

S Weller, SJ Gould, D Valle - Annual review of genomics and …, 2003 - annualreviews.org
The peroxisome biogenesis disorders (PBDs) comprise 12 autosomal recessive
complementation groups (CGs). The multisystem clinical phenotype varies widely in severity …

ATP-Dependent Steps in Peroxisomal Protein Import

HW Platta, J Jeske, N Schmidt… - Annual Review of …, 2024 - annualreviews.org
Peroxisomes are organelles that play a central role in lipid metabolism and cellular redox
homeostasis. The import of peroxisomal matrix proteins by peroxisomal targeting signal …

Ubiquitination of mammalian Pex5p, the peroxisomal import receptor

AF Carvalho, MP Pinto, CP Grou, IS Alencastre… - Journal of Biological …, 2007 - ASBMB
Protein translocation across the peroxisomal membrane requires the concerted action of
numerous peroxins. One central component of this machinery is Pex5p, the cycling receptor …

Members of the E2D (UbcH5) family mediate the ubiquitination of the conserved cysteine of Pex5p, the peroxisomal import receptor

CP Grou, AF Carvalho, MP Pinto, S Wiese… - Journal of Biological …, 2008 - ASBMB
According to current models of peroxisomal biogenesis, newly synthesized peroxisomal
matrix proteins are transported into the organelle by Pex5p. Pex5p recognizes these …