[HTML][HTML] Human disorders of peroxisome metabolism and biogenesis

HR Waterham, S Ferdinandusse… - Biochimica Et Biophysica …, 2016 - Elsevier
Peroxisomes are dynamic organelles that play an essential role in a variety of cellular
catabolic and anabolic metabolic pathways, including fatty acid alpha-and beta-oxidation …

[HTML][HTML] Genetics and molecular basis of human peroxisome biogenesis disorders

HR Waterham, MS Ebberink - … et Biophysica Acta (BBA)-Molecular Basis of …, 2012 - Elsevier
Human peroxisome biogenesis disorders (PBDs) are a heterogeneous group of autosomal
recessive disorders comprised of two clinically distinct subtypes: the Zellweger syndrome …

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 …

[HTML][HTML] Multiple pathways for protein transport to peroxisomes

PK Kim, EH Hettema - Journal of molecular biology, 2015 - Elsevier
Peroxisomes are unique among the organelles of the endomembrane system. Unlike other
organelles that derive most if not all of their proteins from the ER (endoplasmic reticulum) …

Functional role of the AAA peroxins in dislocation of the cycling PTS1 receptor back to the cytosol

HW Platta, S Grunau, K Rosenkranz, W Girzalsky… - Nature cell …, 2005 - nature.com
Peroxisomal import receptors bind their cargo proteins in the cytosol and target them to
docking and translocation machinery at the peroxisomal membrane (reviewed in ref.). The …

Ubiquitination of the peroxisomal import receptor Pex5p is required for its recycling

HW Platta, FE Magraoui, D Schlee, S Grunau… - The Journal of cell …, 2007 - rupress.org
Pex5p, which is the import receptor for peroxisomal matrix proteins harboring a type I signal
sequence (PTS1), is mono-and polyubiquitinated in Saccharomyces cerevisiae. We …

The similarity between N-terminal targeting signals for protein import into different organelles and its evolutionary relevance

M Kunze, J Berger - Frontiers in physiology, 2015 - frontiersin.org
The proper distribution of proteins between the cytosol and various membrane-bound
compartments is crucial for the functionality of eukaryotic cells. This requires the cooperation …

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 …

Peroxisomal matrix protein import: the transient pore model

R Erdmann, W Schliebs - Nature Reviews Molecular Cell Biology, 2005 - nature.com
Peroxisomes import folded, even oligomeric, proteins, which distinguishes the peroxisomal
translocation machinery from the well-characterized translocons of other organelles. How …