Defects in PEX genes impair peroxisome assembly and multiple metabolic pathways
confined to this organelle, thus providing the biochemical and molecular bases of the …

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

Genome-wide association studies (GWAS) have identified thousands of variants associated
with complex traits, but their biological interpretation often remains unclear. Most of these …

Peroxisomes have long been established to play a central role in regulating various
metabolic activities in mammalian cells. These organelles act in concert with mitochondria to …

Peroxisomes contain numerous enzymatic activities that are important for mammalian
physiology. Patients lacking either all peroxisomal functions or a single enzyme or …

Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum disease and
rhizomelic chondrodysplasia punctata are progressive disorders characterized by loss of …

Peroxisomes are components of virtually all eukaryotic cells. While much is known about
peroxisomal matrix protein import, our understanding of how peroxisomal membrane …

Integral peroxisomal membrane proteins (PMPs) are synthesized in the cytoplasm and
imported posttranslationally. Here, we demonstrate that PEX19 binds and stabilizes newly …

PEX19 is a chaperone and import receptor for newly synthesized, class I peroxisomal
membrane proteins (PMPs). PEX19 binds these PMPs in the cytoplasm and delivers them to …

Peroxisomes can arise de novo from the endoplasmic reticulum (ER) via a maturation
process. Peroxisomes can also multiply by fission. We have investigated how these modes …