Mitochondrial dysfunction is a central factor in CNS diseases. mtBiolabs provides patient-derived cell models and cutting-edge technologies to investigate mitochondrial behavior, guide disease-modifying drug (DMD) selection, and quantify neuronal responses.
Mitochondria are central to cellular homeostasis, regulating energy production, redox signaling, and programmed cell death. Their ability to rapidly adapt to metabolic stress depends on a fine balance between biogenesis and removal of damaged organelles. Disruption of this balance is strongly linked to aging and major pathologies, including neurodegenerative diseases1.
Neurons are particularly dependent on mitochondrial function due to their high energy demands and elevated ATP requirements2. Their unique architecture—featuring long axons and complex dendritic networks—requires precise mitochondrial transport and distribution to sustain activity at synapses and distal cellular regions3.
Because neurons are post-mitotic and long-lived, they are especially vulnerable to mitochondrial dysfunction. Impaired mitochondria not only reduce ATP production but also disrupt calcium homeostasis and increase reactive oxygen species (ROS), triggering cellular stress and contributing to neurodegeneration2.
To maintain neuronal health, damaged mitochondria must be repaired or removed through tightly regulated quality control mechanisms, including proteasomal activity, mitochondrial dynamics (fission–fusion), and mitophagy.
Understanding and monitoring these mitochondrial surveillance pathways is therefore critical for advancing research and therapeutic strategies in CNS diseases.
What it is: A technological platform to develop patient-derived cell models to test disease modification through mitochondrial behavior signatures.
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What it is: High-content analysis (HCA) platform for quantitative assessment of mitochondrial DNA.
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What it is: Multiplexed assay platform to evaluate mitochondrial function and drug mechanism of action. The BBS package is available in HTS/single point or dose response studies.
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What it is: Comprehensive profiling of cellular oxidative stress and antioxidant defenses, available in HTS/dose response studies.
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What it is: Patient-derived cell models with identified mitochondrial signatures for specific diseases.
Models available include signatures for:
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All our assays are fully customizable to meet your needs.
Contact us to discuss your studyDiscover how our mitochondrial platforms support different therapeutic areas.
