Establishment of an electrophysiological platform for modeling ALS with regionally-specific human pluripotent stem cell-derived astrocytes and neurons

A Taga, CW Habela, A Johns, S Liu, M O'brien… - JoVE (Journal of …, 2021 - jove.com
Human pluripotent stem cell-derived astrocytes (hiPSC-A) and neurons (hiPSC-N) provide a
powerful tool for modeling Amyotrophic Lateral Sclerosis (ALS) pathophysiology in vitro …

[HTML][HTML] Neuropathogenesis-on-chips for neurodegenerative diseases

S Amartumur, H Nguyen, T Huynh, TS Kim… - Nature …, 2024 - nature.com
Developing diagnostics and treatments for neurodegenerative diseases (NDs) is
challenging due to multifactorial pathogenesis that progresses gradually. Advanced in vitro …

Transgenic and physiological mouse models give insights into different aspects of amyotrophic lateral sclerosis

F De Giorgio, C Maduro, EMC Fisher… - Disease models & …, 2019 - journals.biologists.com
ABSTRACT A wide range of genetic mouse models is available to help researchers dissect
human disease mechanisms. Each type of model has its own distinctive characteristics …

[HTML][HTML] Modeling Neurodegenerative Diseases Using In Vitro Compartmentalized Microfluidic Devices

L Miny, BGC Maisonneuve, I Quadrio… - … in Bioengineering and …, 2022 - frontiersin.org
The human brain is a complex organ composed of many different types of cells
interconnected to create an organized system able to efficiently process information …

[HTML][HTML] Muscle involvement in amyotrophic lateral sclerosis: understanding the pathogenesis and advancing therapeutics

E Duranti, C Villa - Biomolecules, 2023 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a fatal condition characterized by the selective loss of
motor neurons in the motor cortex, brainstem, and spinal cord. Muscle involvement, muscle …

Clinical testing and spinal cord removal in a mouse model for amyotrophic lateral sclerosis (ALS)

R Günther, M Suhr, JC Koch, M Bähr, P Lingor… - JoVE (Journal of …, 2012 - jove.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder resulting in
progressive degeneration of motoneurons. Peak of onset is around 60 years for the sporadic …

[HTML][HTML] Functional skeletal muscle model derived from SOD1-mutant ALS patient iPSCs recapitulates hallmarks of disease progression

A Badu-Mensah, X Guo, CW McAleer, JW Rumsey… - Scientific reports, 2020 - nature.com
Recent findings suggest a pathologic role of skeletal muscle in amyotrophic lateral sclerosis
(ALS) onset and progression. However, the exact mechanism by which this occurs remains …

[HTML][HTML] Use of 3D organoids as a model to study idiopathic form of Parkinson's disease

P Chlebanowska, A Tejchman, M Sułkowski… - International Journal of …, 2020 - mdpi.com
Organoids are becoming particularly popular in modeling diseases that are difficult to
reproduce in animals, due to anatomical differences in the structure of a given organ. Thus …

[HTML][HTML] Engineering a 3D functional human peripheral nerve in vitro using the Nerve-on-a-Chip platform

AD Sharma, L McCoy, E Jacobs, H Willey, JQ Behn… - Scientific reports, 2019 - nature.com
Abstract Development of “organ-on-a-chip” systems for neuroscience applications are
lagging due in part to the structural complexity of the nervous system and limited access of …

Dynamic meta-analysis as a therapeutic prediction tool for amyotrophic lateral sclerosis

CS Mitchell, RH Lee - Amyotrophic lateral sclerosis, 2012 - books.google.com
In this chapter, we present a new method, dynamic meta-analysis, which allows the
examination of the underlying system dynamics of ALS utilizing the wealth of existing …