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Related Concept Videos

Cell Specific Gene Expression01:58

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Multicellular organisms contain a variety of structurally and functionally distinct cell types, but the DNA in all the cells originated from the same parent cells. The differences in the cells can be attributed to the differential gene expression. Liver cells, whose functions include detoxification of blood, production of bile to metabolize fats, and synthesis of proteins essential for metabolism, must express a specific set of genes to perform their functions. Gene expression also varies with...
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Heterotrimeric G proteins are guanine nucleotide-binding proteins. As the name suggests, heterotrimeric G proteins are composed of three subunits: alpha, beta, and gamma. They remain GDP-bound or GTP-bound inside the cells and switch between inactive/active states. The Gα subunit possesses the nucleotide-binding pocket that binds guanine nucleotides and switches between GDP or GTP-bound states. In contrast, the Gꞵ and Gγ subunits are always bound together with high...
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Updated: Mar 12, 2026

Using Fluorescence Activated Cell Sorting to Examine Cell-Type-Specific Gene Expression in Rat Brain Tissue
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ALS-causing mutations differentially affect PGC-1α expression and function in the brain vs. peripheral tissues.

Hanna Bayer1, Kerstin Lang1, Eva Buck1

  • 1Department of Neurology, Ulm University, DE 89081, Germany.

Neurobiology of Disease
|November 8, 2016
PubMed
Summary
This summary is machine-generated.

Amyotrophic lateral sclerosis (ALS) mutations disrupt the PGC-1α metabolic regulator, decreasing it in the brain but increasing it peripherally. Lactate emerges as a key inducer of brain PGC-1α, linking metabolism to neuroprotection in ALS.

Keywords:
Amyotrophic lateral sclerosisFUSLactateLou Gehrig diseasePGC-1αSOD1

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Area of Science:

  • Neuroscience
  • Metabolic pathways
  • Neurodegenerative diseases

Background:

  • Monogenetic forms of ALS provide insights into neurodegenerative mechanisms.
  • Investigating the role of the metabolic regulator PGC-1α (PPAR gamma coactivator)-1α in ALS.
  • Examining the link between metabolic alterations and neurodegeneration in ALS.

Purpose of the Study:

  • To determine if ALS-causing mutations affect the expression and signaling of PGC-1α in the periphery and brain.
  • To elucidate the role of PGC-1α in the metabolic changes associated with ALS.

Main Methods:

  • Analysis of PGC-1α isoforms and target genes in mouse models of familial ALS.
  • Validation of PGC-1α signaling in primary adipocytes and neurons from ALS models.
  • Assessment of PGC-1α signaling in iPS-derived motoneurons from ALS patients with FUS/TLS mutations.

Main Results:

  • ALS mutations (SOD1, FUS/TLS) reduced PGC-1α levels in the CNS but increased them in peripheral tissues.
  • Lactate was identified as a potent inducer of CNS-specific PGC-1α isoforms, promoting neuroprotective genes.
  • Lactate-induced PGC-1α signaling was abolished in motoneurons from ALS patients.

Conclusions:

  • ALS mutations dysregulate PGC-1α, inhibiting CNS-specific isoforms while activating peripheral pathways.
  • Lactate acts as a crucial link between brain metabolism and neuroprotection via the PGC-1α system.
  • Alterations in the PGC-1α system may contribute to metabolic changes and neurodegeneration observed in ALS.