Elucidating cellular mechanisms underlying neurodegeneration

阐明神经变性的细胞机制

基本信息

  • 批准号:
    10435954
  • 负责人:
  • 金额:
    $ 57.57万
  • 依托单位:
  • 依托单位国家:
    美国
  • 项目类别:
  • 财政年份:
    2022
  • 资助国家:
    美国
  • 起止时间:
    2022-06-17 至 2027-05-31
  • 项目状态:
    未结题

项目摘要

Giant axonal neuropathy (GAN) is an early-onset, autosomal recessive neurodegenerative disease that impacts the central and peripheral nervous systems. Pathologically, GAN is characterized by the disorganization and aggregation of intermediate filaments (IF). Formed from self-assembling subunits, the IF network spans the cell from the nucleus to the periphery. In GAN, many cell types show abnormalities in the organization of IF, but neurons clearly bear the brunt of the pathology. Axons swell with the accumulation of neuronal IF, and degenerate to cause the neurological symptoms of GAN. The gene mutated in GAN encodes gigaxonin, a protein that belongs to the BTB/Kelch family of E3 ligase-like adaptor proteins. These proteins typically play a role in ubiquitin-proteasome mediated protein degradation. Based on our own data that GAN degrades neuronal IFs, we hypothesize that neurofilament aggregation creates steric roadblocks in neurites that interfere with intracellular transport of organelles such as mitochondria and lysosomes, resulting in downstream pathology. Furthermore, our preliminary data suggest gigaxonin plays a direct role in autophagy via degradation of other substrates. We hypothesize that the disruption of this critical process exacerbates GAN neuropathology by dysregulation of protein and organellar quality control. This proposal comprehensively tests these models; thus the overall goal of our research is to understand the cellular pathogenesis of GAN with a view to inspiring novel treatment strategies.
巨轴索神经病(GAN)是一种早发的常染色体隐性神经退行性疾病

项目成果

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Puneet Opal其他文献

Puneet Opal的其他文献

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{{ truncateString('Puneet Opal', 18)}}的其他基金

VEGF-Mimetic Supramolecular Nanoparticles for Treating Spinocerebellar Ataxia Type 1
VEGF 模拟超分子纳米颗粒用于治疗 1 型脊髓小脑共济失调
  • 批准号:
    10578485
  • 财政年份:
    2023
  • 资助金额:
    $ 57.57万
  • 项目类别:
Equipment Supplement: Understanding the Cellular Basis of Movement Disorders
设备补充:了解运动障碍的细胞基础
  • 批准号:
    10755946
  • 财政年份:
    2023
  • 资助金额:
    $ 57.57万
  • 项目类别:
Elucidating cellular mechanisms underlying neurodegeneration
阐明神经变性的细胞机制
  • 批准号:
    10647869
  • 财政年份:
    2022
  • 资助金额:
    $ 57.57万
  • 项目类别:
Developing novel treatment strategies for Spinocerebellar ataxia type 1
开发 1 型脊髓小脑共济失调的新治疗策略
  • 批准号:
    9226821
  • 财政年份:
    2016
  • 资助金额:
    $ 57.57万
  • 项目类别:
Understanding the cellular basis of Movement Disorders
了解运动障碍的细胞基础
  • 批准号:
    8876831
  • 财政年份:
    2013
  • 资助金额:
    $ 57.57万
  • 项目类别:
Understanding the cellular basis of Movement Disorders
了解运动障碍的细胞基础
  • 批准号:
    8631893
  • 财政年份:
    2013
  • 资助金额:
    $ 57.57万
  • 项目类别:
Understanding the Cellular Basis of Movement Disorders
了解运动障碍的细胞基础
  • 批准号:
    10630308
  • 财政年份:
    2013
  • 资助金额:
    $ 57.57万
  • 项目类别:
Understanding the Cellular Basis of Movement Disorders
了解运动障碍的细胞基础
  • 批准号:
    10403448
  • 财政年份:
    2013
  • 资助金额:
    $ 57.57万
  • 项目类别:
Understanding the cellular basis of Movement Disorders
了解运动障碍的细胞基础
  • 批准号:
    8719191
  • 财政年份:
    2013
  • 资助金额:
    $ 57.57万
  • 项目类别:
Understanding the Cellular Basis of Movement Disorders
了解运动障碍的细胞基础
  • 批准号:
    10160963
  • 财政年份:
    2013
  • 资助金额:
    $ 57.57万
  • 项目类别:

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