Mitochondrial membrane lipid remodeling in pathophysiology: A new target for diet and therapeutic interventions

Monteiro, João P.; Oliveira, Paulo J.; Jurado, Amália S.

doi:10.1016/j.plipres.2013.06.002

Cited by 81 publications

(55 citation statements)

References 240 publications

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“…MAMs are enriched in cholesterol and sphingolipids, increasing the thickness of these membranes (Monteiro et al 2013). They incorporate a distinct set of proteins that includes the inositol 1,4,5-trisphosphate receptor (IP3R), originating from the ER, as well as the mitochondrial channel VDAC, the chaperones grp75 and sigma-1 receptor, the sorting protein PACS-2, the mitochondrial fission factor Fis1, the ER protein Bap31, the mitofusin Mfn2, the OMM protein PTPIP51 and the vesicle-associated membrane protein B (VAPB) (Schon and Area-Gomez 2013; Stoica et al 2014).…”

Section: Mitochondria-associated Membranes: a Lipid Point Of Viewmentioning

confidence: 99%

Mitochondrial lipids in neurodegeneration

et al. 2016

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Mitochondrial dysfunction is a common feature of many neurodegenerative diseases, including proteinopathies such as Alzheimer’s or Parkinson’s disease, which are characterized by the deposition of aggregated proteins in the form of insoluble fibrils or plaques. The distinct molecular processes that eventually result in mitochondrial dysfunction during neurodegeneration are well studied but still not fully understood. However, defects in mitochondrial fission and fusion, mitophagy, oxidative phosphorylation and mitochondrial bioenergetics have been linked to cellular demise. These processes are influenced by the lipid environment within mitochondrial membranes as, besides membrane structure and curvature, recruitment and activity of different proteins also largely depend on the respective lipid composition. Hence, the interaction of neurotoxic proteins with certain lipids and the modification of lipid composition in different cell compartments, in particular mitochondria, decisively impact cell death associated with neurodegeneration. Here, we discuss the relevance of mitochondrial lipids in the pathological alterations that result in neuronal demise, focussing on proteinopathies.

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Section: Mitochondria-associated Membranes: a Lipid Point Of Viewmentioning

confidence: 99%

Mitochondrial lipids in neurodegeneration

et al. 2016

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“…The particular roles of individual PL classes are associated with characteristic FA compositions that confer specific properties related to membrane fluidity and functions (Zabelinskii et al 1999). Therefore, not only changes in PL class composition, but also alterations in PL FA compositions would modify their molecular properties and their roles in membrane functions (Shigenaga et al 1994;Chicco and Sparagna 2007;Crimi and Esposti 2011;Monteiro et al 2013).…”

Section: Introductionmentioning

confidence: 99%

Dietary fatty acids affect mitochondrial phospholipid compositions and mitochondrial gene expression of rainbow trout liver at different ages

et al. 2014

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Mitochondria are among the first responders to various stressors that challenge the homeostasis of cells and organisms. Mitochondrial decay is generally associated with impairment in the organelle bioenergetics function and increased oxidative stress, and it appears that deterioration of mitochondrial inner membrane phospholipids (PL), particularly cardiolipin (CL), and accumulation of mitochondrial DNA (mtDNA) mutations are among the main mechanisms involved in this process. In the present study, liver mitochondrial membrane PL compositions, lipid peroxidation and mtDNA gene expression were analyzed in rainbow trout fed three diets with the same base formulation but with lipid supplied either by fish oil (FO), rapeseed oil (RO) or a high DHA oil (DHA) during six weeks. Specifically, two feeding trials were performed using fish from the same population of two ages (1 and 3 years), and PL class compositions of liver mitochondria, fatty acid composition of individual PL classes, TBARS content and mtDNA expression were determined. Dietary fatty acid composition strongly affected mitochondrial membrane composition from trout liver but observed changes did not fully reflect the diet, particularly when it contained high DHA. The changes were PL specific, CL being particularly resistant to changes in DHA. Some significant differences observed in expression of mtDNA with diet may suggest long-term dietary effects in mitochondrial gene expression which could affect electron transport chain function. All the changes were influenced by fish age, which could be related to the different growth rates observed between 1- and 3-year-old trout but that could also indicate age-related changes in the ability to maintain structural homeostasis of mitochondrial membranes

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“…Other novel therapeutic approaches include targeting the mitochondrial membrane and its complex lipids46; replacing nucleosides in mtDNA depletion disorders that are associated with nucleoside deficiency47; and gene therapy approaches including recoding mtDNA-encoded genes to be expressed in the nuclear genome (allotopic gene expression), ‘conventional’ viral vector-mediated nuclear-targeted gene therapies; and selective destruction of mutant mtDNA using restriction endonuclease technologies (see ref 5). …”

Section: Management Of Mitochondrial Disordersmentioning

confidence: 99%

Recognition, investigation and management of mitochondrial disease

Davison

Rahman

2017

Arch Dis Child

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Mitochondria are dynamic organelles present in virtually all human cells that are needed for a multitude of cellular functions, including energy production, control of cell apoptosis and numerous biochemical catabolic and synthetic pathways that are critical for cellular health. Primary mitochondrial disorders are a group of greater than 200 single gene defects arising from two genomes (nuclear and mitochondrial) leading to mitochondrial dysfunction, and are associated with extremely heterogeneous phenotypes. Neuromuscular features predominate, but often with multisystem involvement. Clinical suspicion of a mitochondrial disorder should prompt multipronged investigation with biochemical and molecular genetic studies. Recent wide-scale adoption of next-generation sequencing approaches has led to a rapid increase in the number of disease genes. The advances in unravelling the genetic landscape of mitochondrial diseases have not yet been matched by progress in developing effective therapies, and the mainstay of care remains supportive therapies in a multidisciplinary team setting.

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Mitochondrial membrane lipid remodeling in pathophysiology: A new target for diet and therapeutic interventions

Cited by 81 publications

References 240 publications

Mitochondrial lipids in neurodegeneration

Mitochondrial lipids in neurodegeneration

Dietary fatty acids affect mitochondrial phospholipid compositions and mitochondrial gene expression of rainbow trout liver at different ages

Recognition, investigation and management of mitochondrial disease

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