Huntington’s Disease and Stem Cells

2017 ◽  
pp. 115-144
Author(s):  
Karen Ring ◽  
Robert O’Brien ◽  
Ningzhe Zhang ◽  
Lisa M. Ellerby
Keyword(s):  
Stem Cells ◽  
Huntington's Disease ◽  
Huntington’S Disease

Bone marrow mesenchymal stem cells can improve the motor function of a Huntington's disease rat model

2011 ◽  
Vol 33 (3) ◽  
pp. 331-337 ◽  
Author(s):  
Yufeng Jiang ◽  
Hailong Lv ◽  
Shanshan Huang ◽  
Huiping Tan ◽  
Yinong Zhang ◽  
...  
Keyword(s):  
Stem Cells ◽  
Bone Marrow ◽  
Mesenchymal Stem Cells ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Motor Function ◽  
Rat Model ◽  
Marrow Mesenchymal Stem Cells

scholarly journals Transplants of Adult Mesenchymal and Neural Stem Cells Provide Neuroprotection and Behavioral Sparing in a Transgenic Rat Model of Huntington's Disease

Stem Cells ◽  
2014 ◽  
Vol 32 (2) ◽  
pp. 500-509 ◽  
Author(s):  
Julien Rossignol ◽  
Kyle Fink ◽  
Kendra Davis ◽  
Steven Clerc ◽  
Andrew Crane ◽  
...  
Keyword(s):  
Stem Cells ◽  
Neural Stem Cells ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rat Model ◽  
Transgenic Rat ◽  
Transgenic Rat Model

The Use of Stem Cells in Treating Huntington’s Disease

2014 ◽  
pp. 20-55
Author(s):  
K Fink ◽  
A Crane ◽  
J Rossignol ◽  
L Lescaudron ◽  
G Dunbar
Keyword(s):  
Stem Cells ◽  
Huntington's Disease ◽  
Huntington’S Disease

scholarly journals Human Neural Stem Cells Differentiate and Integrate, Innervating Implanted zQ175 Huntington’s Disease Mouse Striatum

2021 ◽  
Author(s):  
Sandra M. Holley ◽  
Jack C. Reidling ◽  
Carlos Cepeda ◽  
Alice Lau ◽  
Cindy Moore ◽  
...  
Keyword(s):  
Stem Cells ◽  
Neural Stem Cells ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Pyramidal Neurons ◽  
Neurodegenerative Disorder ◽  
Functional Integration ◽  
Behavioral Deficits ◽  
Human Neural Stem Cells ◽  
Neuronal Populations

AbstractHuntington’s disease (HD), a genetic neurodegenerative disorder, primarily impacts the striatum and cortex with progressive loss of medium-sized spiny neurons (MSNs) and pyramidal neurons, disrupting cortico-striatal circuitry. A promising regenerative therapeutic strategy of transplanting human neural stem cells (hNSCs) is challenged by the need for long-term functional integration. We previously described that hNSCs transplanted into the striatum of HD mouse models differentiated into electrophysiologically active immature neurons, improving behavior and biochemical deficits. Here we show that 8-month implantation of hNSCs into the striatum of zQ175 HD mice ameliorates behavioral deficits, increases brain-derived neurotrophic factor (BDNF) and reduces mutant Huntingtin (mHTT) accumulation. Patch clamp recordings, immunohistochemistry and electron microscopy demonstrates that hNSCs differentiate into diverse neuronal populations, including MSN- and interneuron-like cells. Remarkably, hNSCs receive synaptic inputs, innervate host neurons, and improve membrane and synaptic properties. Overall, the findings support hNSC transplantation for further evaluation and clinical development for HD.

scholarly journals Genomic Analysis Reveals Disruption of Striatal Neuronal Development and Therapeutic Targets in Human Huntington’s Disease Neural Stem Cells

2015 ◽  
Vol 5 (6) ◽  
pp. 1023-1038 ◽  
Author(s):  
Karen L. Ring ◽  
Mahru C. An ◽  
Ningzhe Zhang ◽  
Robert N. O’Brien ◽  
Eliana Marisa Ramos ◽  
...  
Keyword(s):  
Stem Cells ◽  
Neural Stem Cells ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neuronal Development ◽  
Therapeutic Targets ◽  
Genomic Analysis

scholarly journals Recent Overview of the Use of iPSCs Huntington’s Disease Modeling and Therapy

2020 ◽  
Vol 21 (6) ◽  
pp. 2239 ◽  
Author(s):  
Maria Csobonyeiova ◽  
Stefan Polak ◽  
Lubos Danisovic
Keyword(s):  
Stem Cells ◽  
Stem Cell ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Modeling ◽  
Cell Types ◽  
Cag Repeats ◽  
Neural Cells ◽  
Behavioral Impairment ◽  
Cell Based Therapy

Huntington’s disease (HD) is an inherited, autosomal dominant, degenerative disease characterized by involuntary movements, cognitive decline, and behavioral impairment ending in death. HD is caused by an expansion in the number of CAG repeats in the huntingtin gene on chromosome 4. To date, no effective therapy for preventing the onset or progression of the disease has been found, and many symptoms do not respond to pharmacologic treatment. However, recent results of pre-clinical trials suggest a beneficial effect of stem-cell-based therapy. Induced pluripotent stem cells (iPSCs) represent an unlimited cell source and are the most suitable among the various types of autologous stem cells due to their patient specificity and ability to differentiate into a variety of cell types both in vitro and in vivo. Furthermore, the cultivation of iPSC-derived neural cells offers the possibility of studying the etiopathology of neurodegenerative diseases, such as HD. Moreover, differentiated neural cells can organize into three-dimensional (3D) organoids, mimicking the complex architecture of the brain. In this article, we present a comprehensive review of recent HD models, the methods for differentiating HD–iPSCs into the desired neural cell types, and the progress in gene editing techniques leading toward stem-cell-based therapy.

scholarly journals Extracts of Adipose Derived Stem Cells Slows Progression in the R6/2 Model of Huntington's Disease

PLoS ONE ◽  
2013 ◽  
Vol 8 (4) ◽  
pp. e59438 ◽  
Author(s):  
Wooseok Im ◽  
Jaejun Ban ◽  
Jiyeon Lim ◽  
Mijung Lee ◽  
Soon-Tae Lee ◽  
...  
Keyword(s):  
Stem Cells ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Adipose Derived Stem Cells
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