Charcot-marie-tooth Modeling
Charcot-marie-tooth Modeling - Related Videos
07:43
Modeling Charcot-Marie-Tooth Disease In Vitro by Transfecting Mouse Primary Motoneurons
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Cited by 4 •
2019
The goal of this technique is to prepare a highly enriched culture of primary motoneurons (MNs) from murine spinal cord. To evaluate the consequences of mutations causing MN diseases, we describe here the isolation of these isolated MNs and their transfection by magnetofection.
02:57
Analyzing Tooth Germ and Trigeminal Ganglia Interactions Using a Microfluidic Co-culture System
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2025
This video demonstrates the utilization of a microfluidic device to study the interactions between tooth germ pulp and trigeminal ganglia. It showcases the placement of tissues inside the microfluidic device and provides a detailed visualization of trigeminal neurite growth toward the tooth germ through the device's microgrooves. Additionally, it highlights the role of tooth-derived molecular factors in promoting or inhibiting neurite extension into the tooth germ pulp, which is crucial for...
01:21
Tooth Anatomy
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2024
The human tooth enables us to eat a variety of foods, speak clearly, and even aid in shaping our faces. Teeth are composed of various elements that work together. Here's a detailed look at the anatomy of a human tooth. The Crown, Neck, and Root The visible part of the tooth is referred to as the crown. It's covered by enamel, the hardest substance in the human body. The crown is uniquely shaped for each type of tooth, allowing for different functions such as cutting, tearing, or grinding food.
08:01
Analysis of Developing Tooth Germ Innervation Using Microfluidic Co-culture Devices
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Cited by 10 •
2015
Co-cultures represent a valuable method to study the interactions between nerves and target tissues and organs. Microfluidic systems allow co-culturing ganglia and whole developing organs or tissues in different culture media, thus representing a valuable tool for the in vitro study of the crosstalk between neurons and their targets.
07:09
In situ Compressive Loading and Correlative Noninvasive Imaging of the Bone-periodontal Ligament-tooth Fibrous Joint
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Cited by 17 •
2014
In this study, the use of an in situ loading device coupled with micro-X-ray computed tomography for fibrous joint biomechanics will be discussed. Experimental readouts identifiable with an overall change in joint biomechanics will include: 1) reactionary force vs. displacement, i.e. tooth displacement within the alveolar socket and its reactionary response to loading, 2) three-dimensional (3D) spatial configuration and morphometrics, i.e. geometric relationship of the tooth with the alveolar...
FAQs
The modeled mutation and the cell type carrying it determine which disease process becomes visible. Neuronal systems can reveal changes linked to axonal transport, whereas Schwann-cell systems can expose abnormalities associated with myelination. Whole-organism models add the opportunity to examine how these disturbances relate to progressive axonal degeneration and movement-related phenotypes.
These processes describe different levels of peripheral nerve failure that can be connected within one model. Altered myelination may affect nerve signaling, disrupted axonal transport may compromise neuronal maintenance, and progressive axonal degeneration may help explain worsening movement. Examining them together lets investigators relate molecular changes to structural and functional consequences rather than treating each feature in isolation.
Model choice determines the kind of evidence available. Patient-derived cells and induced pluripotent stem cell models support investigation of mutation-associated changes in human cellular systems, while organoids provide another experimental scale. Genetically modified animals extend analysis to whole-organism effects, making comparisons across levels useful for linking cellular abnormalities with disease features.
Computational systems add a complementary way to represent Charcot-Marie-Tooth disease mechanisms alongside experimental models. Their value lies in connecting disease-associated genetic changes with molecular mechanisms and observed clinical features, while experimental systems examine those changes in cells or organisms. Together, these approaches can support disease classification and organize relationships among mutations, nerve abnormalities, and functional impairment.
Researchers can tailor a model by selecting the disease-associated genetic change, the biological level of interest, and the system that best represents it. Options described for this work include patient-derived cells, induced pluripotent stem cell models, organoids, genetically modified animals, and computational systems. This selection determines whether analysis emphasizes cellular mechanisms, whole-organism effects, or connections to clinical features.
Analysis can focus on structural and functional changes in peripheral nerves, including disrupted myelination, altered axonal transport, and progressive axonal degeneration. Models may also help connect these findings with weakness, sensory loss, and impaired gait. Such outcomes provide a basis for examining how a mutation-associated molecular disturbance is expressed across nerve biology and clinically relevant function.
Researchers apply these models to several goals: classifying disease forms, identifying biomarkers, and evaluating potential therapies. Patient-derived and stem-cell systems can help investigate mutation-specific cellular effects, whereas animal models can place those effects in the context of a whole organism. In neuroscience, this multilevel evidence supports links between molecular mechanisms and peripheral nerve function.