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Basic Science and Pathogenesis.

Matthew J Rosene1, Abraham Lopez2, Adeeb Ahmad2

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Summary
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Cysteine string protein-α (CSPα) mutations cause cell-specific aggregation and disrupt proteostasis, impacting Alzheimer's disease (AD) pathways. This study investigates CSPα's role in AD pathogenesis and microglial function.

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Area of Science:

  • Neuroscience
  • Cell Biology
  • Genetics

Background:

  • DNAJC5 encodes cysteine string protein-α (CSPα), an endolysosomal cochaperone involved in synaptic integrity and the autophagy/lysosomal pathway (ALP).
  • Elevated CSPα expression in microglia suggests cell-type-specific roles in brain proteostasis, particularly relevant to Alzheimer's disease (AD).

Purpose of the Study:

  • To investigate CSPα-dependent proteostasis.
  • To explore the role of CSPα in Alzheimer's disease (AD) pathogenesis.

Main Methods:

  • Generated transcriptome and proteomic data from human cell lines (neuronal, astrocyte, microglial) overexpressing wild-type (WT) and mutant CSPα.
  • Analyzed patient-derived iPSCs with mutant CSPα and CRISPR-corrected controls, differentiated into cortical neurons.
  • Integrated proteomic data from human brain tissue of CSPα mutation carriers and healthy controls.

Main Results:

  • Mutant CSPα carriers exhibit CSPα aggregates and autophagic dysfunction, replicating findings in AD brain tissue.
  • Proteomic and transcriptomic analyses reveal altered ALP proteins, inflammation-associated proteins (especially in microglia), and differentially expressed genes related to microglia and interferon response.
  • Mutant CSPα expression leads to protein aggregation, depalmitoylation, and altered autophagic flux in neuronal and microglial cell lines.

Conclusions:

  • Mutant CSPα induces cell-type-specific aggregation and dysregulates proteostatic pathways, with implications for AD-related proteinopathies.
  • Further multi-omics analysis will clarify cell-type-specific CSPα-associated neurodegenerative pathways.
  • Future research will elucidate CSPα's precise regulation of AD proteostasis.