PEX16: a multifaceted regulator of peroxisome biogenesis
نویسندگان
چکیده
منابع مشابه
PEX16: a multifaceted regulator of peroxisome biogenesis
PEROXISOMES ARE FORMED BY TWO DISTINCT PATHWAYS the growth and fission of mature peroxisomes and de novo synthesis at the endoplasmic reticulum (ER). While many of the molecular mechanisms underlying these two pathways remain to be elucidated, it is generally accepted that their relative contribution to peroxisome formation may vary depending on the species, cell type and/or physiological statu...
متن کاملIdentification of an unusual variant peroxisome biogenesis disorder caused by mutations in the PEX16 gene.
BACKGROUND Zellweger syndrome spectrum disorders are caused by mutations in any of at least 12 different PEX genes. This includes PEX16, which encodes an integral peroxisomal membrane protein involved in peroxisomal membrane assembly. PEX16-defective patients have been reported to have a severe clinical presentation. Fibroblasts from these patients displayed a defect in the import of peroxisoma...
متن کاملDe Novo Peroxisome Biogenesis in Penicillium Chrysogenum Is Not Dependent on the Pex11 Family Members or Pex16
We have analyzed the role of the three members of the Pex11 protein family in peroxisome formation in the filamentous fungus Penicillium chrysogenum. Two of these, Pex11 and Pex11C, are components of the peroxisomal membrane, while Pex11B is present at the endoplasmic reticulum. We show that Pex11 is a major factor involved in peroxisome proliferation. We also demonstrate that P. chrysogenum ce...
متن کاملPeroxisome biogenesis and positioning.
Plant peroxisomes are extremely dynamic, moving and undergoing changes of shape in response to metabolic and environmental signals. Matrix proteins are imported via one of two import pathways, depending on the targeting signal within the protein. Each pathway has a specific receptor but utilizes common membrane-bound translocation machinery. Current models invoke receptor recycling, which may i...
متن کاملPeroxisome biogenesis and human peroxisome-deficiency disorders
Peroxisome is a single-membrane-bounded ubiquitous organelle containing a hundred different enzymes that catalyze various metabolic pathways such as β-oxidation of very long-chain fatty acids and synthesis of plasmalogens. To investigate peroxisome biogenesis and human peroxisome biogenesis disorders (PBDs) including Zellweger syndrome, more than a dozen different complementation groups of Chin...
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ژورنال
عنوان ژورنال: Frontiers in Physiology
سال: 2013
ISSN: 1664-042X
DOI: 10.3389/fphys.2013.00241