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Lipidomics and Transcriptomics in Neurological Diseases
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Sphingolipids in neurodegenerative diseases.

Xueyang Pan1,2, Debdeep Dutta1,2, Shenzhao Lu1,2

  • 1Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, United States.

Frontiers in Neuroscience
|March 6, 2023
PubMed
Summary

Sphingolipid metabolism defects are implicated in numerous neurodegenerative diseases (NDDs), including Parkinson's and ALS. Studies in fruit flies and patient samples reveal key cellular and metabolic alterations, offering therapeutic insights for these conditions.

Keywords:
DrosophilaParkinson’s diseaseceramideslysosomemitochondrianeurodegenerationsphingolipids

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Area of Science:

  • Neuroscience
  • Molecular Biology
  • Genetics

Background:

  • Neurodegenerative diseases (NDDs) are characterized by progressive neuronal dysfunction.
  • Emerging evidence links sphingolipid metabolism disruptions to a wide range of NDDs.
  • These include lysosomal storage diseases (LSDs), hereditary spastic paraplegia (HSP), and Parkinson's disease (PD).

Purpose of the Study:

  • To summarize research on sphingolipid metabolism defects in NDDs.
  • To highlight findings from *Drosophila melanogaster* models and patient samples.
  • To identify implicated organelles, affected cell types, and potential therapeutic strategies.

Main Methods:

  • Review of studies utilizing *Drosophila melanogaster* models of NDDs.
  • Analysis of patient samples for sphingolipid alterations.
  • Examination of cellular and organelle dysfunction in affected NDDs.

Main Results:

  • Elevated ceramide levels are a common hallmark in various NDD models.
  • Specific defects in sphingolipid metabolism pathways are identified.
  • Early cellular and organelle dysfunctions are characterized in affected cell types.

Conclusions:

  • Sphingolipid metabolism is a critical factor in the pathogenesis of diverse NDDs.
  • Fruit fly models provide valuable insights into NDD mechanisms.
  • Understanding these metabolic defects opens avenues for novel therapeutic interventions.