Phospholipids: Identification and Implication in Muscle Pathophysiology

Abstract

Phospholipids (PLs) are amphiphilic molecules that were essential for life to become cellular. PLs have not only a key role in compartmentation as they are the main components of membrane, but they are also involved in cell signaling, cell metabolism, and even cell pathophysiology. Considered for a long time to simply be structural elements of membranes, phospholipids are increasingly being viewed as sensors of their environment and regulators of many metabolic processes. After presenting their main characteristics, we expose the increasing methods of PL detection and identification that help to understand their key role in life processes. Interest and importance of PL homeostasis is growing as pathogenic variants in genes involved in PL biosynthesis and/or remodeling are linked to human diseases. We here review diseases that involve deregulation of PL homeostasis and present a predominantly muscular phenotype.

Lipid Biosynthesis Perturbation Impairs Endoplasmic Reticulum-Associated Degradation

“…Perturbed membrane lipid composition has been implicated in multiple diseases, including nonalcoholic fatty liver disease (LI et al 2006;ARENDT et al 2013), obesity and type II diabetes (KIM et al 2016), muscular dystrophy (MITSUHASHI AND NISHINO 2011), and cardiomyopathies (BARGUI et al 2021). Our results suggest alterations in lipid profiles associated with these disease states are likely to impair ERAD.…”

“…2013), obesity and type II diabetes (K im et al . 2016), muscular dystrophy (M itsuhashi and N ishino 2011), and cardiomyopathies (B argui et al . 2021).…”

“…Taken together, our results indicate that altered lipid homeostasis broadly and profoundly impairs ER proteostasis. Several metabolic, muscular, cardiac, and neurodegenerative diseases are associated with perturbed lipid synthesis (LI et al 2006;MITSUHASHI AND NISHINO 2011;ARENDT et al 2013;KOSICEK AND HECIMOVIC 2013;KIM et al 2016;BARGUI et al 2021). Altered lipid homeostasis may impair ER protein degradation in individuals with these disorders.…”

Research Progress on Nanoparticles-Based CRISPR/Cas9 System for Targeted Therapy of Tumors

“…Lipids are a diverse group of naturally occurring or synthetic organic compounds which include fatty acids and their derivatives [ 68 ]. Amphiphilic phospholipids are a major component of cell membranes and liposomes [ 69 ]. Therefore, liposomes as three-dimensional spherical structures that encapsulate hydrophilic or hydrophobic molecules can enter cells by endocytosis [ 70 ].…”

Understanding the nitrolipidome: From chemistry to mass spectrometry and biological significance of modified complex lipids

“…Phospholipids (PLs) and triacylglycerides (TAGs) are complex lipids commonly present in cell membranes and in lipoproteins. These complex lipids are structural components in membranes and signaling molecules (e.g, PLs) but also energy storage (e.g., TAGs) [1,2]. Both PLs and TAGs are susceptible to be modified by reactive oxygen species (ROS) and reactive nitrogen species (RNS).…”