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Cited by 282Mitochondrial Function and PathologyLipid metabolism and biosynthesisFatty Acid Research and Health

Cardiolipin, Mitochondria, and Neurological Disease

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Falabella, Micol, Vernon, Hilary J., Hanna, Michael G., Claypool, Steven M., Pitceathly, Robert D.S.·2021·Trends in Endocrinology & Metabolism
DOI 10.1016/j.tem.2021.01.006PMC PMC8277580
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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.

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  • Metabolic interventions such as insulin sensitizers, mitochondrial stabilizers, and AMPK modulators aim to correct energy deficits that heighten synaptic vulnerability in Alzheimer's disease.

    “In parallel, metabolic interventions such as insulin sensitizers [180], mitochondrial stabilizers [181], and AMPK modulators aim to correct energy deficits that heighten synaptic vulnerability.”

    From Toward a Unified Framework in Molecular Neurobiology of Alzheimer’s Disease: Revisiting the Pathophysiological Hypotheses (2026) · cites this paper as [181]

    The Management and Treatment Strategies section describes a mitochondrial-targeted compound proposed to stabilize cardiolipin and improve oxidative phosphorylation. The Alzheimer’s Disease section links reduced cardiolipin to mitochondrial synaptic dysfunction in a mouse model. The paper does not establish that mitochondrial stabilizers correct energy deficits or reduce synaptic vulnerability in Alzheimer’s disease. (LLM, conf=0.82)

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Authors
Falabella, M., Vernon, H. J., Hanna, M. G., Claypool, S. M., & Pitceathly, R. D. S.
Affiliations
  • UCL Queen Square Institute of Neurology (GB)
  • Johns Hopkins University (US)
  • National Hospital for Neurology and Neurosurgery (GB)
Corresponding author
Pitceathly, Robert D.S.
ORCID
Venue
Trends in Endocrinology & Metabolism
Publisher
Elsevier BV
Published
2021-04
Volume
32
Issue
4
Pages
224-237
ISSN
1043-2760 (ISSN-L)
DOI
10.1016/j.tem.2021.01.006
PMC
PMC8277580
OpenAlex
W3133026938
Cited by
282
References
3
Citations by year
2026: 51 · 2025: 69 · 2024: 68 · 2023: 43 · 2022: 37 · 2021: 14
FWCI
4.32
Type
journal article
Language
en
Open access
Licence
Locations
  • Trends in Endocrinology and Metabolism (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • UCL Discovery (University College London) (submittedVersion)
Funders
Medical Research Council
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Lipid metabolism and biosynthesis
  • Health Sciences › Nursing › Nutrition and Dietetics › Fatty Acid Research and Health
Keywords
cardiolipin, mitochondria, neurodegenerative diseases, lipid homeostasis, Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, traumatic brain injury, acyl chain composition, biomarkers, neurogenesis, mitochondrial dysfunction
MeSH terms
Cardiolipins, Central Nervous System, Humans, Mitochondria, Neurons, Neurodegenerative Diseases

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