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Understanding the Structure of TFG and Its Role in the Nervous System

Ziheng Zhang (Ph.D.)
4.9/5 (17383 ratings)
Description:Mutations in the tropomyosin receptor kinase-fused gene (TFG) are associated with several neurodegenerative diseases presenting distinct clinical features. The effect of pathological TFG mutations can vary greatly, depending on the domain impacted. Specifically, mutations in the N-terminal PB1 domain and coiled-coil domain of TFG are associated with Hereditary spastic paraplegias, an early onset, progressive condition leading to lower limb weakness and spasticity. Remarkably, HSP patients with mutations in the PB1 domain appear to show a distinct set of phenotypes than those with coiled-coil domain mutations. The structural basis of these distinct sets of phenotypes is unknown. In this study, I describe a model of the structure of TFG and how it forms oligomers, determined by X-ray crystallography, Cryo-electron microscopy, and light scattering analysis. Light scattering analysis shows that HSP associated pathological mutations weaken the TFG oligomerization interface and destabilize the oligomer, ultimately leading to neurodegeneration. Importantly, the impact of one of the PB1 domain mutations, p.R22W, is neuron specific, and distinct from the impact of another previously described variant. Altogether, these studies expand our understanding of TFG oligomerization and its role in the nervous system.We have made it easy for you to find a PDF Ebooks without any digging. And by having access to our ebooks online or by storing it on your computer, you have convenient answers with Understanding the Structure of TFG and Its Role in the Nervous System. To get started finding Understanding the Structure of TFG and Its Role in the Nervous System, you are right to find our website which has a comprehensive collection of manuals listed.
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Pages
0
Format
PDF, EPUB & Kindle Edition
Publisher
Release
2024
ISBN
tFgNadyPaWEC

Understanding the Structure of TFG and Its Role in the Nervous System

Ziheng Zhang (Ph.D.)
4.4/5 (1290744 ratings)
Description: Mutations in the tropomyosin receptor kinase-fused gene (TFG) are associated with several neurodegenerative diseases presenting distinct clinical features. The effect of pathological TFG mutations can vary greatly, depending on the domain impacted. Specifically, mutations in the N-terminal PB1 domain and coiled-coil domain of TFG are associated with Hereditary spastic paraplegias, an early onset, progressive condition leading to lower limb weakness and spasticity. Remarkably, HSP patients with mutations in the PB1 domain appear to show a distinct set of phenotypes than those with coiled-coil domain mutations. The structural basis of these distinct sets of phenotypes is unknown. In this study, I describe a model of the structure of TFG and how it forms oligomers, determined by X-ray crystallography, Cryo-electron microscopy, and light scattering analysis. Light scattering analysis shows that HSP associated pathological mutations weaken the TFG oligomerization interface and destabilize the oligomer, ultimately leading to neurodegeneration. Importantly, the impact of one of the PB1 domain mutations, p.R22W, is neuron specific, and distinct from the impact of another previously described variant. Altogether, these studies expand our understanding of TFG oligomerization and its role in the nervous system.We have made it easy for you to find a PDF Ebooks without any digging. And by having access to our ebooks online or by storing it on your computer, you have convenient answers with Understanding the Structure of TFG and Its Role in the Nervous System. To get started finding Understanding the Structure of TFG and Its Role in the Nervous System, you are right to find our website which has a comprehensive collection of manuals listed.
Our library is the biggest of these that have literally hundreds of thousands of different products represented.
Pages
0
Format
PDF, EPUB & Kindle Edition
Publisher
Release
2024
ISBN
tFgNadyPaWEC
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