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Cited by 37Amyotrophic Lateral Sclerosis ResearchAlzheimer's disease research and treatmentsAmyloidosis: Diagnosis, Treatment, Outcomes

Disease-modifying effects of metabolic perturbations in ALS/FTLD

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Jawaid, Ali, Khan, Romesa, Polymenidou, Magdalini, Schulz, Paul E.·2018·Molecular Neurodegeneration
DOI 10.1186/s13024-018-0294-0PMC PMC6278047
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Abstract

Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are two fatal neurodegenerative disorders with considerable clinical, pathological and genetic overlap. Both disorders are characterized by the accumulation of pathological protein aggregates that contain a number of proteins, most notably TAR DNA binding protein 43 kDa (TDP-43). Surprisingly, recent clinical studies suggest that dyslipidemia, high body mass index, and type 2 diabetes mellitus are associated with better clinical outcomes in ALS. Moreover, ALS and FTLD patients have a significantly lower incidence of cardiovascular disease, supporting the idea that an unfavorable metabolic profile may be beneficial in ALS and FTLD. The two most widely studied ALS/FTLD models, super-oxide dismutase 1 (SOD1) and TAR DNA binding protein of 43 kDA (TDP-43), reveal metabolic dysfunction and a positive effect of metabolic strategies on disease onset and/or progression. In addition, molecular studies reveal a role for ALS/FTLD-associated proteins in the regulation of cellular and whole-body metabolism. Here, we systematically evaluate these observations and discuss how changes in cellular glucose/lipid metabolism may result in abnormal protein aggregations in ALS and FTLD, which may have important implications for new treatment strategies for ALS/FTLD and possibly other neurodegenerative conditions.

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  • Previous studies have suggested that a high circulating level of glucose and a higher energy balance can potentially counteract the energy deficit of ALS related to aberrant aggregations of proteins in neurons.

    “Previous studies have suggested several pathways potentially underlying the observed inverse association between diabetes mellitus type 2 and ALS risk; for instance, a high circulating level of glucose and a higher energy balance can potentially counteract the energy deficit of ALS related to aberrant aggregations of proteins in neurons [35].”

    From Lifestyle and medical conditions in relation to ALS risk and progression—an introduction to the Swedish ALSrisc Study (2024) · cites this paper as [35]

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Authors
Jawaid, A., Khan, R., Polymenidou, M., & Schulz, P. E.
Affiliations
  • University of Zurich (CH)
  • Lahore University of Management Sciences (PK)
  • The University of Texas Health Science Center at Houston (US)
Corresponding author
Jawaid, Ali
ORCID
Venue
Molecular Neurodegeneration
Publisher
Springer Science and Business Media LLC
Published
2018-12
Volume
13
Issue
1
Pages
63-63
ISSN
1750-1326 (ISSN-L)
DOI
10.1186/s13024-018-0294-0
PMC
PMC6278047
OpenAlex
W2902480371
Cited by
37
References
12
Citations by year
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FWCI
0.83
Type
journal article
Language
en
Open access
Licence
Locations
  • Molecular Neurodegeneration (publishedVersion, cc-by)
  • Europe PMC (PubMed Central) (publishedVersion, other-oa)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • Zurich Open Repository and Archive (University of Zurich) (acceptedVersion, cc-by)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
  • Repository for Publications and Research Data (ETH Zurich) (submittedVersion, cc-by)
  • Zurich Open Repository and Archive (University of Zurich) (submittedVersion, cc-by)
  • ETH Zürich Research Collection (cc-by)
  • Zurich Open Repository and Archive (University of Zurich)
Topics
  • Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
  • Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
Keywords
amyotrophic lateral sclerosis, frontotemporal lobar degeneration, metabolic dysfunction, TDP-43, SOD1, protein aggregation, dyslipidemia, type 2 diabetes, body mass index, cardiovascular disease, glucose metabolism, lipid metabolism, disease-modifying therapy, neurodegeneration
MeSH terms
Amyotrophic Lateral Sclerosis, Animals, Inclusion Bodies, Diabetes Mellitus, Type 2, Humans, Mutation, Frontotemporal Lobar Degeneration, Frontotemporal Dementia

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

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