Ceramide and the mitochondrial respiratory chain.

Biochimie

Monique and Jacques Roboh Department of Genetic Research, Hadassah-Hebrew University Medical Center, Jerusalem, Israel; Department of Genetics and Metabolic Diseases, Hadassah-Hebrew University Medical Center, Jerusalem, Israel. Electronic address:

Published: May 2014

Ceramide is a group of sphingolipids found in cell membranes, composed of a sphingoid base linked to a fatty acid of varying chain length. Initially regarded as purely structural components, this group of molecules is now recognized as a key signaling and regulatory elements in cell biology. Ceramide species differing in acyl chain length, with distinct biophysical properties, execute distinct functions and effects. Some of these modulate mitochondrial function and oxidative phosphorylation (OXPHOS). Certain ceramides were associated with decreased mitochondrial respiratory chain (MRC) activity, increased reactive oxygen species (ROS) production and oxidative stress, mitochondrial outer membrane permeabilization (MOMP), reduced mitochondrial membrane potential, mitophagy and apoptosis. In this review we aim to summarize the most relevant findings linking ceramide to mitochondria. The physiological significance of synthetic short and naturally occurring long chain ceramides in modulating mitochondrial function with emphasis on the MRC will be discussed.

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.biochi.2013.07.027DOI Listing

Publication Analysis

Top Keywords

mitochondrial respiratory
8
respiratory chain
8
chain length
8
mitochondrial function
8
chain
5
mitochondrial
5
ceramide
4
ceramide mitochondrial
4
chain ceramide
4
ceramide group
4

Similar Publications

Mitochondrial disease and epilepsy in children.

Front Neurol

January 2025

Department of Pediatric Neurology, Children's Medical Center, First Hospital of Jilin University, Changchun, China.

Mitochondria is the cell's powerhouse. Mitochondrial disease refers to a group of clinically heterogeneous disorders caused by dysfunction in the mitochondrial respiratory chain, often due to mutations in mitochondrial DNA (mtDNA) or nuclear DNA (nDNA) that encodes mitochondrial proteins. This dysfunction can lead to a variety of clinical phenotypes, particularly affecting organs with high energy demands, such as the brain and muscles.

View Article and Find Full Text PDF

Nitric oxide (NO) is a ubiquitous signaling molecule known to modulate various physiological processes, with specific implications in skeletal muscle and broader applications in exercise performance. This review focuses on the modulation of skeletal muscle function, mitochondrial adaptation and function, redox state by NO, and the effect of nitrate supplementation on exercise performance. In skeletal muscle function, NO is believed to increase the maximal shortening velocity and peak power output of muscle fibers.

View Article and Find Full Text PDF

Neutrophil extracellular traps in tumor metabolism and microenvironment.

Biomark Res

January 2025

Department of Respiratory Disease, Daping Hospital, Army Medical University, Chongqing, China.

Neutrophil extracellular traps (NETs) are intricate, web-like formations composed of DNA, histones, and antimicrobial proteins, released by neutrophils. These structures participate in a wide array of physiological and pathological activities, including immune rheumatic diseases and damage to target organs. Recently, the connection between NETs and cancer has garnered significant attention.

View Article and Find Full Text PDF

Lithium compromises the bioenergetic reserve of cardiomyoblasts mitochondria.

J Bioenerg Biomembr

January 2025

Institute of Molecular Physiology and Genetics, Centre of Biosciences of the Slovak Academy of Sciences, Dubravska cesta 9, Bratislava, 840 05, Slovakia.

Lithium is used in the long-term treatment of bipolar disorder, exhibiting a beneficial effect on the neuronal cells. The concentration of lithium in the blood serum can vary and can easily approach a level that is related to cardiotoxic adverse effects. This is due to its narrow therapeutic index.

View Article and Find Full Text PDF

Homozygous missense variant in causes early-onset neurodegeneration, leukoencephalopathy and autoinflammation.

J Med Genet

January 2025

Division of Clinical and Metabolic Genetics, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada

Biallelic pathogenic variants in cause a fatal autosomal recessive multisystem disorder characterized by recurrent autoinflammation, hypomyelination, progressive neurodegeneration, microcephaly, failure to thrive, liver dysfunction, respiratory chain defects and accumulation of glycogen in skeletal muscle. No missense variants in have been reported to date.We report a 6-year-old boy with microcephaly, global developmental delays, lower limb spasticity with hyperreflexia, epilepsy, abnormal brain MRI, failure to thrive, recurrent fevers and transaminitis.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!