TY - JOUR
T1 - THE PHYSIOLOGICAL FUNCTIONS OF HUMAN PEROXISOMES
AU - Wanders, Ronald J. A.
AU - Baes, Myriam
AU - Ribeiro, Daniela
AU - Ferdinandusse, Sacha
AU - Waterham, Hans R.
N1 - Funding Information: Throughout the years, research on PBDs in the R.J.A.W., S.F., and H.R.W. laboratories has been supported by grants from ZonMW, NWO, the “Prinses Beatrix Fonds,” the “Hersenstichting,” Stichting MetaKids, Stichting Stofwisselkracht, the 6th Framework Program of the European Union, and the Marie Curie Initial Training Networks action FP7 and Horizon2020. M.B. was supported by grants from the KU Leuven (C14/18/088) and from the Research Foundation-Flanders (FWO G0A8619N). D.R. was supported by the Portuguese Foundation for Science and Technology (FCT), PTDC/BIA-CEL/31378/2017 and CEECIND/03747/2017, under the scope of the Operational Program “Competitiveness and internationalization,” in its FEDER/FNR component, and by the European Union through the Horizon 2020 program: H2020-WIDESPREAD-2020-5 ID-952373. Publisher Copyright: © 2023 the American Physiological Society.
PY - 2023/1/1
Y1 - 2023/1/1
N2 - Peroxisomes are subcellular organelles that play a central role in human physiology by catalyzing a range of unique metabolic functions. The importance of peroxisomes for human health is exemplified by the existence of a group of usually severe diseases caused by an impairment in one or more peroxisomal functions. Among others these include the Zellweger spectrum disorders, X-linked adrenoleukodystrophy, and Refsum disease. To fulfill their role in metabolism, peroxisomes require continued interaction with other subcellular organelles including lipid droplets, lysosomes, the endoplasmic reticulum, and mitochondria. In recent years it has become clear that the metabolic alliance between peroxisomes and other organelles requires the active participation of tethering proteins to bring the organelles physically closer together, thereby achieving efficient transfer of metabolites. This review intends to describe the current state of knowledge about the metabolic role of peroxi-somes in humans, with particular emphasis on the metabolic partnership between peroxisomes and other organelles and the consequences of genetic defects in these processes. We also describe the biogenesis of peroxisomes and the consequences of the multiple genetic defects therein. In addition, we discuss the functional role of peroxisomes in different organs and tissues and include relevant information derived from model systems, notably peroxisomal mouse models. Finally, we pay particular attention to a hitherto underrated role of peroxisomes in viral infections.
AB - Peroxisomes are subcellular organelles that play a central role in human physiology by catalyzing a range of unique metabolic functions. The importance of peroxisomes for human health is exemplified by the existence of a group of usually severe diseases caused by an impairment in one or more peroxisomal functions. Among others these include the Zellweger spectrum disorders, X-linked adrenoleukodystrophy, and Refsum disease. To fulfill their role in metabolism, peroxisomes require continued interaction with other subcellular organelles including lipid droplets, lysosomes, the endoplasmic reticulum, and mitochondria. In recent years it has become clear that the metabolic alliance between peroxisomes and other organelles requires the active participation of tethering proteins to bring the organelles physically closer together, thereby achieving efficient transfer of metabolites. This review intends to describe the current state of knowledge about the metabolic role of peroxi-somes in humans, with particular emphasis on the metabolic partnership between peroxisomes and other organelles and the consequences of genetic defects in these processes. We also describe the biogenesis of peroxisomes and the consequences of the multiple genetic defects therein. In addition, we discuss the functional role of peroxisomes in different organs and tissues and include relevant information derived from model systems, notably peroxisomal mouse models. Finally, we pay particular attention to a hitherto underrated role of peroxisomes in viral infections.
KW - mouse models
KW - peroxisomal disorders
KW - peroxisomal metabolism
KW - peroxisome biogenesis
KW - viral infection
UR - http://www.scopus.com/inward/record.url?scp=85140992027&partnerID=8YFLogxK
U2 - https://doi.org/10.1152/physrev.00051.2021
DO - https://doi.org/10.1152/physrev.00051.2021
M3 - Review article
C2 - 35951481
SN - 0031-9333
VL - 103
SP - 957
EP - 1024
JO - Physiological Reviews
JF - Physiological Reviews
IS - 1
ER -