Neurological Disease

Neurodisease Application
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In vitro neural models are powerful tools to study neural function and complex disorders of the human nervous system. Neurodegenerative diseases, like Alzheimer’s, Parkinson’s, or ALS, result in the progressive degeneration of healthy neurons while neurodevelopmental disorders, like autism, Down syndrome, or schizophrenia affect brain development and ultimately impact function. Both types of neurological disease require the ability to study neural networks and how they change over time.

Maestro Pro and Edge microelectrode array (MEA) technology is uniquely suited for this purpose, with noninvasive measurements that can be performed over days and months while preserving the complex structure and communication of neuronal networks. With the added ability to culture and measure multiple lines simultaneously, MEA is an ideal platform for studying disease-in-a-dish models.

Discover how a mutation impacts neural function

Alzheimer’s disease and neuroinflammation

Chronic neuroinflammation impairs microglial clearance of amyloid beta (Aβ), contributing to cognitive decline in Alzheimer’s disease. In this study, scientists examined the impact of Aβ42 oligomers on neurons and investigated if peripheral immune cells can protect synapses from damage. Maestro MEA data demonstrated that Aβ42 oligomers reduced synaptic integrity and contributed to altered electrical activity after only 24 hours and showed that co-culture with activated, bone-derived macrophages resulted in synaptic preservation—findings that mirror phenotypes observed in vivo. [Li et al. 2020.]

Spike shape recorded from neurons changing with addition of immune cells


Fragile X

In an iPSC-derived model of Fragile X syndrome, the cultures were first characterized by a significant reduction in FMR1 and FMRP expression, relative to an isogenic control. The corresponding network electrophysiology phenotype featured an increase in excitability (weighted mean firing rate) at 21+ days in culture. Co-culture control and Fragile X neurons at different ratios revealed that only approximately 20% healthy neurons were necessary to restore activity to control levels [Liu et al. 2018]. 

Fragile X spontaneous activity


Burning Man Syndrome

Neurons expressing a specific mutation associated with inherited erythromelagia, a chronic pain syndrome characterized by a burning sensation in response to heat, were cultured on the MEA and treated with carbamazepine which reduced their sensitivity to temperature increases. Two patients with the same mutation were then treated with carbamazepine and reported a reduction in pain duration when compared to a placebo [Mis et al. 2018].

Neural activity map of burning man pain syndrome on MEA plate


Parkinson’s Disease

Data obtained from the Maestro contributed toward better understanding of the genetic and nongenetic causes of Parkinson’s disease. In a study involving identical twins, one with Parkinson’s and the other without, researchers were able to compare the impaired network activity of the Parkinson's twin to the synchronous network of the healthy twin [Woodard et al. 2014].

Results from studies of Parkinsons Disease
Assay Steps
Neuro disease protocol

Getting started with Maestro Pro and Edge couldn't be easier. Culture your neurons in an Axion multiwell MEA plate (Day 0).  Load the MEA plate into the Maestro MEA system at the desired recording times and begin recording.  Analyze the neural activity in the MEA plate label-free and in real-time with AxIS Navigator Neural Module software