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- W2984160161 abstract "In the past decade, studies on MCSs have led to the identification of new MCSs and the molecular constitution of MCSs. Disturbed MCSs between peroxisomes and other cell organelles can cause disease. Such diseases by definition affect several cell organelles and may be termed ‘organelle interaction diseases’. Peroxisome–organelle contact also plays a role in an expanding range of diseases that are not primarily peroxisomal. Advanced understanding of MCSs requires systems-level analysis and opens new horizons to pathomechanisms and interventions. Peroxisomes share extensive metabolic connections with other cell organelles. Membrane contact sites (MCSs) establish and maintain such interactions, and they are vital for organelle positioning and motility. In the past few years peroxisome interactions and MCSs with other cellular organelles have been explored extensively, resulting in the identification of new MCSs, the tethering molecules involved, and their functional characterization. Defective tethering and compartmental communication can lead to pathological conditions that can be termed ‘organelle interaction diseases’. We review peroxisome–organelle interactions in mammals and summarize the most recent knowledge of mammalian peroxisomal organelle contacts in health and disease. Peroxisomes share extensive metabolic connections with other cell organelles. Membrane contact sites (MCSs) establish and maintain such interactions, and they are vital for organelle positioning and motility. In the past few years peroxisome interactions and MCSs with other cellular organelles have been explored extensively, resulting in the identification of new MCSs, the tethering molecules involved, and their functional characterization. Defective tethering and compartmental communication can lead to pathological conditions that can be termed ‘organelle interaction diseases’. We review peroxisome–organelle interactions in mammals and summarize the most recent knowledge of mammalian peroxisomal organelle contacts in health and disease. loss of the myelin sheath along the length of the internode or near the paranodal area. Demyelination results in slow conduction of nerve impulses, causing neurological disease. also known as subacute necrotizing encephalomyelopathy, a neurometabolic multiorgan disorder caused by defects in mitochondrial function. cells located in the connective tissue surrounding the seminiferous tubules in the testicle. They produce testosterone, the male sex hormone responsible for the development of secondary sex characteristics. mitofusin 2 (MFN2) is a mitochondrial outer-membrane GTPase that participates in mitochondrial fusion and contributes to the maintenance of mitochondrial network. Mutations in its coding gene MFN2 are implicated in Charcot–Marie–Tooth disease (CMT). paranodal and juxtaparanodal regions lie adjacent to the nodes of Ranvier – myelin-free regions along the myelinated axons. These regions are highly enriched in ion channels, allowing them to participate in the exchange of ions required for action potential generation. encode peroxins or peroxisomal biogenesis factors. Deficiencies in PEX proteins are associated with peroxisomal biogenesis disorders (PBDs). phospholipids in which the acyl chain in position 1 is linked to glycerol by a vinyl-ether linkage. The first two steps of plasmalogen synthesis are located in peroxisomes. stop-codon suppression leading to continuation of translation beyond a stop codon owing to recoding of the stop codon as a sense codon. Functional translational readthrough adds functional extensions to proteins. an evolutionary conserved cellular stress response cascade caused by the accumulation of misfolded proteins in the endoplasmic reticulum (ER). The UPR mitigates stress by directing the cell to either survival or apoptosis. conserved integral membrane proteins of the ER. Humans have two VAPs, VAPA and VAPB. VAPs are implicated in lipid transport and homeostasis, membrane trafficking, and the UPR. VAPs interact with client proteins on other organelles that contain FFAT." @default.
- W2984160161 created "2019-11-22" @default.
- W2984160161 creator A5053722819 @default.
- W2984160161 creator A5081825305 @default.
- W2984160161 date "2020-02-01" @default.
- W2984160161 modified "2023-10-17" @default.
- W2984160161 title "Staying in Healthy Contact: How Peroxisomes Interact with Other Cell Organelles" @default.
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