Human peroxisome biogenesis disorders (PBDs) are a heterogeneous group of autosomal
recessive disorders comprised of two clinically distinct subtypes: the Zellweger syndrome …

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 …

Peroxisomes are ubiquitous organelles whose dysfunction causes fatal human diseases.
Most peroxisomal enzymes are imported from the cytosol by the receptor PEX5, which …

The peroxisomal protein import machinery differs fundamentally from known translocons
(endoplasmic reticulum, mitochondria, chloroplasts, bacteria) as it allows membrane …

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 …

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) …

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 …

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 …

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 …

Peroxisomes constitute a dynamic compartment in all nucleated cells. They fulfill diverse
metabolic tasks in response to environmental changes and cellular demands. This …