Issue Cover (May 2018)

Front cover:Background: Mitochondria and lipid metabolism regulate each other in the skeletal muscle. Altered lipid transport, metabolism and storage influence the tissue bioenergetics ultimately leading to cardiac and neurological diseases. Lipid storage disorders (LSDs) are neurometabolic disorders which display intramuscular lipid accumulation and impaired mitochondrial bioenergetics in the muscle, leading to progressive myopathy. Main findings: To understand the relationship between lipid metabolism and mitochondria, we carried out morphological and biochemical analysis of mitochondrial function in muscle biopsies of LSD patients. Altered mitochondrial structure, impaired fatty acid and respiratory metabolism along with increased membrane permeability and elevated lipolysis entail mitochondrial dysfunction in LSD. Implication: Many differentially regulated mitochondrial proteins in LSD are linked with other human diseases indicating that mitochondrial protection via targeted drugs could be a treatment modality in LSD and related metabolic diseases. Image Content: The cover image shows the transverse section of skeletal muscle tissue from a case of LSD, stained with Oli‐red‐O. Prominent multiple red deposits correspond to intracellular accumulation of lipids (magnification: X20).Read the full article ‘ Mitochondrial dysfunction in human skeletal muscle biopsies of lipid storage disorder’ by B. Debashree, M. Kumar, T. S. Keshava Prasad, A. Natarajan, R. Christopher, A. Nalini, P. S. Bindu, N. Gayathri, M. M. Srinivas Bharath ( J. Neurochem . 2018, vol. 145 (4), pp. 323–341) ondoi: 10.1111/jnc.14318