In order to point out the conserved mechanism, we discuss our findings in the context of the working models of peroxisomal biogenesis and pexophagy in yeasts and mammals. Therefore, we conclude that Pex1 is directly and essentially involved in peroxisomal matrix protein import, and that the PEX1 deletion-induced pexophagy is not responsible for the defect in peroxisomal function. In the present study, we can show that the specific block of PEX1 deletion-induced pexophagy does not restore peroxisomal matrix protein import or the peroxisomal function in beta-oxidation in yeast. Thus, the observed matrix protein import defect would not be caused by a lack of Pex1 activity, but rather by enhanced removal of peroxisomal membranes via pexophagy. The resulting working model proposed that Pex1 may not be essential for matrix protein import at all, but rather for the prevention of pexophagy. Recent work from patient cells that contain the Pex1(G843D) point mutant suggested that the inhibition of the lysosome, and therefore the block of pexophagy, was beneficial for peroxisomal function. PBDs are caused by mutations in the peroxisomal biogenesis factors, which are required for the correct compartmentalization of peroxisomal matrix enzymes. This spectrum of autosomal recessive metabolic disorders is characterized by defective peroxisome assembly and impaired peroxisomal functions. The important physiologic role of peroxisomes is shown by the occurrence of peroxisomal biogenesis disorders (PBDs) in humans. Authors may use MDPI'sĮnglish editing service prior to publication or during author revisions. Submitted papers should be well formatted and use good English. For details about the APC please see here. There is an Article Processing Charge (APC) for publication in this Please visit the Instructions for Authors page before submitting a manuscript. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. All manuscripts are thoroughly refereed through a single-blind peer-review process. Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website. Research articles, review articles as well as short communications are invited. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website.
All submissions that pass pre-check are peer-reviewed. Manuscripts can be submitted until the deadline. Once you are registered, click here to go to the submission form.
Manuscripts should be submitted online at by registering and logging in to this website. Both original research articles and review papers that stimulate discussion are welcome. Potential topics include, but are not limited to, the molecular, biochemical, and cellular mechanisms that control peroxisome dynamics and functions in various organisms and disease states. This Special Issue aims to provide a forum for researchers around the world to share their latest findings and views on how peroxisomes communicate with their environment to support cellular functions. Dysregulation of any of these processes has been demonstrated to impact organismal fitness and survival. This is thought to occur via vesicular traffic, membrane contact sites, and membrane transport proteins. To maintain their function and dynamics, these organelles must exchange substrates, metabolites, and signaling molecules with other subcellular compartments. Peroxisomes are remarkably dynamic organelles whose functions can differ substantially across different species, life stages, and environmental conditions. After being overlooked for a long time, this organelle has now moved into the spotlight of cell biology, biomedical research, and biotechnology. In 2019, it will be 65 years since the Swedish doctoral student Johannes Rhodin described, for the first time, the existence of special organelles, currently known as peroxisomes, in mouse kidney cells.
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