Mitochondrial dysfunction in human skeletal muscle biopsies of lipid storage disorder

• Published in: Journal of neurochemistry Vol. 145; no. 4; pp. 323 - 341
• Main Authors: Debashree, Bandopadhyay, Kumar, Manish, Keshava Prasad, Thottethodi Subrahmanya, Natarajan, Archana, Christopher, Rita, Nalini, Atchayaram, Bindu, Parayil Sankaran, Gayathri, Narayanappa, Srinivas Bharath, Muchukunte Mukunda
• Format: Journal Article
• Language: English
• Published: England Blackwell Publishing Ltd 01.05.2018
• Subjects: Adolescent; Adult; Biochemical analysis; Bioenergetics; Biopsy; Cell morphology; Coronary artery disease; Cristae; Cytology; Disorders; Fatty acids; Female; Heart diseases; Histochemistry; Histology; human; Humans; Inflammation; Insulin; Lipid metabolism; Lipid Metabolism, Inborn Errors - pathology; Lipid Metabolism, Inborn Errors - physiopathology; lipid storage; Lipids; Lipolysis; Lysergide; Male; Medical treatment; Membrane permeability; Metabolic disorders; Metabolism; Mitochondria; Mitochondria, Muscle - metabolism; Mitochondria, Muscle - pathology; Mitochondria, Muscle - ultrastructure; Mitochondrial DNA; Molecular chains; Muscle, Skeletal - pathology; Muscle, Skeletal - physiopathology; Muscle, Skeletal - ultrastructure; Muscles; Musculoskeletal system; Myopathy; Neurological diseases; Protein structure; Proteins; proteomics; Skeletal muscle; Spectrometry; Storage; Young Adult; lipid storage; proteomics; myopathy; human; mitochondria

Mitochondria regulate the balance between lipid metabolism and storage in the skeletal muscle. Altered lipid transport, metabolism and storage influence the bioenergetics, redox status and insulin signalling, contributing to cardiac and neurological diseases. Lipid storage disorders (LSDs) are neuro...