[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 …
recessive disorders comprised of two clinically distinct subtypes: the Zellweger syndrome …
Lipid Droplets and Peroxisomes: Key Players in Cellular Lipid Homeostasis or A Matter of Fat—Store 'em Up or Burn 'em Down
SD Kohlwein, M Veenhuis, IJ van der Klei - Genetics, 2013 - academic.oup.com
Lipid droplets (LDs) and peroxisomes are central players in cellular lipid homeostasis: some
of their main functions are to control the metabolic flux and availability of fatty acids (LDs and …
of their main functions are to control the metabolic flux and availability of fatty acids (LDs and …
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 …
Most peroxisomal enzymes are imported from the cytosol by the receptor PEX5, which …
The peroxisomal importomer constitutes a large and highly dynamic pore
M Meinecke, C Cizmowski, W Schliebs, V Krüger… - Nature cell …, 2010 - nature.com
The peroxisomal protein import machinery differs fundamentally from known translocons
(endoplasmic reticulum, mitochondria, chloroplasts, bacteria) as it allows membrane …
(endoplasmic reticulum, mitochondria, chloroplasts, bacteria) as it allows membrane …
Pexophagy: a model for selective autophagy
K Germain, PK Kim - International journal of molecular sciences, 2020 - mdpi.com
The removal of damaged or superfluous organelles from the cytosol by selective autophagy
is required to maintain organelle function, quality control and overall cellular homeostasis …
is required to maintain organelle function, quality control and overall cellular homeostasis …
[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) …
organelles that derive most if not all of their proteins from the ER (endoplasmic reticulum) …
Peroxisomes are required for in vivo nitric oxide accumulation in the cytosol following salinity stress of Arabidopsis plants
FJ Corpas, M Hayashi, S Mano, M Nishimura… - Plant …, 2009 - academic.oup.com
Peroxisomes are unique organelles involved in multiple cellular metabolic pathways. Nitric
oxide (NO) is a free radical active in many physiological functions under normal and stress …
oxide (NO) is a free radical active in many physiological functions under normal and stress …
Genetic classification and mutational spectrum of more than 600 patients with a Zellweger syndrome spectrum disorder
MS Ebberink, PAW Mooijer, J Gootjes, J Koster… - Human …, 2011 - Wiley Online Library
The autosomal recessive Zellweger syndrome spectrum (ZSS) disorders comprise a main
subgroup of the peroxisome biogenesis disorders and can be caused by mutations in any of …
subgroup of the peroxisome biogenesis disorders and can be caused by mutations in any of …
PEX5 protein binds monomeric catalase blocking its tetramerization and releases it upon binding the N-terminal domain of PEX14
MO Freitas, T Francisco, TA Rodrigues… - Journal of biological …, 2011 - ASBMB
Newly synthesized peroxisomal matrix proteins are targeted to the organelle by PEX5. PEX5
has a dual role in this process. First, it acts as a soluble receptor recognizing these proteins …
has a dual role in this process. First, it acts as a soluble receptor recognizing these proteins …
Import of proteins into the peroxisomal matrix
S Hasan, HW Platta, R Erdmann - Frontiers in physiology, 2013 - frontiersin.org
Peroxisomes constitute a dynamic compartment in all nucleated cells. They fulfill diverse
metabolic tasks in response to environmental changes and cellular demands. This …
metabolic tasks in response to environmental changes and cellular demands. This …