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Updated: Jun 16, 2026

Efficient and Scalable Production of Full-length Human Huntingtin Variants in Mammalian Cells using a Transient Expression System
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Efficient and Scalable Production of Full-length Human Huntingtin Variants in Mammalian Cells using a Transient Expression System

Published on: December 10, 2021

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可溶性突变的亨廷丁在亨廷顿病的早期人类病原体驱动

Andrés Miguez1,2,3,4,5,6, Cinta Gomis7,8,9,10,11, Cristina Vila7,8,9,10,11

  • 1Laboratory of Stem Cells and Regenerative Medicine, Department of Biomedical Sciences, Faculty of Medicine and Health Sciences, University of Barcelona, Barcelona, Spain. andres.miguez@vhir.org.

Cellular and molecular life sciences : CMLS
|August 3, 2023
PubMed
概括

亨廷顿病 (HD) 涉及突变的亨廷丁蛋白损害大脑细胞. 可溶性mHTT准器官,外体细胞传播疾病,导致条状神经元死亡.

关键词:
细胞移植 细胞移植疾病建模 疾病建模细胞外囊泡中的细胞外囊泡.诱导的多能干细胞干细胞神经退行发生神经退行.寡合体是一种寡合体.

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Fractionation for Resolution of Soluble and Insoluble Huntingtin Species

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相关概念视频

Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...