2. Academic Validation
  3. Cardiolipin, Mitochondria, and Neurological Disease

Cardiolipin, Mitochondria, and Neurological Disease

  • Trends Endocrinol Metab. 2021 Apr;32(4):224-237. doi: 10.1016/j.tem.2021.01.006.
Micol Falabella 1,  Hilary J Vernon 2,  Michael G Hanna 3,  Steven M Claypool 4,  Robert D S Pitceathly 5
Affiliations

Affiliations

  • 1 Department of Neuromuscular Diseases, University College London Queen Square Institute of Neurology, London, UK.
  • 2 Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
  • 3 Department of Neuromuscular Diseases, University College London Queen Square Institute of Neurology and The National Hospital for Neurology and Neurosurgery, London, UK.
  • 4 Department of Physiology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
  • 5 Department of Neuromuscular Diseases, University College London Queen Square Institute of Neurology and The National Hospital for Neurology and Neurosurgery, London, UK. Electronic address: r.pitceathly@ucl.ac.uk.
PMID: 33640250 DOI: 10.1016/j.tem.2021.01.006
Abstract

Over the past decade, it has become clear that lipid homeostasis is central to cellular metabolism. Lipids are particularly abundant in the central nervous system (CNS) where they modulate membrane fluidity, electric signal transduction, and synaptic stabilization. Abnormal lipid profiles reported in Alzheimer's disease (AD), Parkinson's disease (PD), amyotrophic lateral sclerosis (ALS), and traumatic brain injury (TBI), are further support for the importance of lipid metablism in the nervous system. Cardiolipin (CL), a mitochondria-exclusive phospholipid, has recently emerged as a focus of neurodegenerative Disease Research. Aberrant CL content, structure, and localization are linked to impaired neurogenesis and neuronal dysfunction, contributing to aging and the pathogenesis of several neurodegenerative diseases, such as AD and PD. Furthermore, the highly tissue-specific acyl chain composition of CL confers it significant potential as a biomarker to diagnose and monitor the progression in several neurological diseases. CL also represents a potential target for pharmacological strategies aimed at treating neurodegeneration. Given the equipoise that currently exists between CL metabolism, mitochondrial function, and Neurological Disease, we review the role of CL in nervous system physiology and monogenic and neurodegenerative disease pathophysiology, in addition to its potential application as a biomarker and pharmacological target.

Keywords

cardiolipin; lipids; mitochondria; mitochondrial disease; nervous system; neurodegeneration.

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