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 …

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

The peroxisome biogenesis disorders (PBD) are a heterogeneous group of autosomal
recessive disorders in which peroxisome assembly is impaired, leading to multiple …

To investigate peroxisome assembly and human peroxisome biogenesis disorders (PBDs)
such as Zellweger syndrome, thirteen different complementation groups (CGs) of Chinese …

The peroxisome-biogenesis disorders (PBDs) are a group of genetically heterogeneous,
lethal diseases that are characterized by neuronal, hepatic, and renal abnormalities; severe …

Summary Peroxisome-biogenesis disorders (PBDs), including Zellweger syndrome (ZS), are
autosomal recessive diseases caused by a deficiency in peroxisome assembly as well as by …

Peroxisome biogenesis disorders in the Zellweger syndrome spectrum (PBD-ZSS) are
caused by defects in at least 12 PEX genes required for normal organelle assembly. Clinical …

Zellweger cerebro-hepato-renal syndrome is a severe congenital disorder associated with
defective peroxisomal biogenesis. At least 23 PEX genes have been reported to be …

Peroxisome biogenesis disorders (PBD) are a heterogeneous group of autosomal recessive
neurodegenerative disorders that affect multiple organ systems. Approximately 80% of PBD …