peroxisomes are single membrane bound organelles present in a wide variety of eukaryotes from yeast to human, have different functions, two of which are well conserved, i.e. hydrogen peroxide decomposition and fatty acid beta-oxidation. the process of peroxisome biogenesis can be divided into distinct steps including peroxisome membrane assembly, import of matrix proteins and peroxisome prolife...

we searched for novel chinese hamster ovary (cho) cell mutants defective in peroxisome biogenesis by an improved method using peroxisome targeting sequence (pts) of pex3p (amino acid residues 1–40)-fused enhanced green fluorescent protein (egfp). from mutagenized tkaeg3(1–40) cells, the wild-type cho-k1 stably expressing rat pex2p and of rat pex3p(1–40)-egfp, numerous cell colonies resistant to...

in this presentation, we review briefly on biogenesis of peroxisomes with regard to the recent findings on biogenesis. peroxisomes perform a range of various functions as oxidation of very long chain fatty acids, generation and removal of hydrogen peroxide, while peroxisomes have also been implicated in β-oxidation of aromatic and cyclic compounds, synthesis of plasmalogens, isoprenoids, lysine...

BACKGROUND Zellweger spectrum disorders (ZSDs) are multisystem genetic disorders caused by a lack of functional peroxisomes, due to mutations in one of the PEX genes, encoding proteins involved in peroxisome biogenesis. The phenotypic spectrum of ZSDs ranges from an early lethal form to much milder presentations. In cultured skin fibroblasts from mildly affected patients, peroxisome biogenesis ...

Background Rhizomelic chondrodysplasia punctata (RCDP) type 1 is among of the rare autosomal recessive peroxisome biogenesis disorders caused by mutations in the PEX7 gene. RCDP patients with the classic form of RCDP1 do not live more than 10- year. Materials and Methods In the present study, a two-year-old girl with skeletal abnormalities and dysmorphic facial appearance is reported to be suf...

in this presentation, we review briefly on biogenesis of peroxisomes with regard to the recent findings on biogenesis. peroxisomes perform a range of various functions as oxidation of very long chain fatty acids, generation and removal of hydrogen peroxide, while peroxisomes have also been implicated in β-oxidation of aromatic and cyclic compounds, synthesis of plasmalogens, isoprenoids, lysine...

We searched for novel Chinese hamster ovary (CHO) cell mutants defective in peroxisome biogenesis by an improved method using peroxisome targeting sequence (PTS) of Pex3p (amino acid residues 1–40)-fused enhanced green fluorescent protein (EGFP). From mutagenized TKaEG3(1–40) cells, the wild-type CHO-K1 stably expressing rat Pex2p and of rat Pex3p(1–40)-EGFP, numerous cell colonies resistant to...

Zellweger syndrome (ZS) is a rare autosomal recessive inherited disorder within the spectrum of peroxisome biogenesis disorders. It is a progressive and fatal disorder with multiple congenital anomalies. There may be some challenges for anesthesiologists in patients with ZS. We report the anesthetic management of an infant with ZS undergoing closure of patent ductus arteriosus and pulmonary art...

PEX13 is an integral membrane protein on the peroxisome that regulates peroxisomal matrix protein import during peroxisome biogenesis. Mutations in PEX13 and other peroxin proteins are associated with Zellweger syndrome spectrum (ZSS) disorders, a subtype of peroxisome biogenesis disorder characterized by prominent neurological, hepatic, and renal abnormalities leading to neonatal death. The la...