ArticleProceedings of the National Academy of Sciences of the United States of America2009
Neural transplants in patients with Huntington's disease undergo disease-like neuronal degeneration.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 76 papers.
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Who cites it
76 citing papers in PubMed, 191 citations in OpenAlex.
- The long-term safety and efficacy of bilateral transplantation of human fetal striatal tissue in patients with mild to moderate Huntington's disease.Journal of neurology, neurosurgery, and psychiatry · 2013Trial
- The Evolving Landscape of Stem Cell Therapies for Huntington's Disease.Molecular diagnosis & therapy · 2026Review
- Advancements in surgical treatments for Huntington disease: From pallidotomy to experimental therapies.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2024Review
- Evidence of mutant huntingtin and tau-related pathology within neuronal grafts in Huntington's disease cases.Neurobiology of disease · 2024Article
- Neural Cells for Neurodegenerative Diseases in Clinical Trials.Stem cells translational medicine · 2023Review
- Translating cell therapies for neurodegenerative diseases: Huntington's disease as a model disorder.Brain : a journal of neurology · 2022Article
- Human iPSC-derived neural precursor cells differentiate into multiple cell types to delay disease progression following transplantation into YAC128 Huntington's disease mouse model.Cell proliferation · 2021Article
- Structural insight into transmissive mutant huntingtin species by correlative light and electron microscopy and cryo-electron tomography.Biochemical and biophysical research communications · 2021Article
- Cell therapy in Huntington's disease: Taking stock of past studies to move the field forward.Stem cells (Dayton, Ohio) · 2021Review
- Molecular Components of Store-Operated Calcium Channels in the Regulation of Neural Stem Cell Physiology, Neurogenesis, and the Pathology of Huntington's Disease.Frontiers in cell and developmental biology · 2021Review
- Insulin-like growth factor 2 (IGF2) protects against Huntington's disease through the extracellular disposal of protein aggregates.Acta neuropathologica · 2020Article
- Huntington disease: new insights into molecular pathogenesis and therapeutic opportunities.Nature reviews. Neurology · 2020Review
- Deconstructing Neurogenesis, Transplantation and Genome-Editing as Neural Repair Strategies in Brain Disease.Frontiers in cell and developmental biology · 2020Review
- Is the Immunological Response a Bottleneck for Cell Therapy in Neurodegenerative Diseases?Frontiers in cellular neuroscience · 2020Review
- Rhes travels from cell to cell and transports Huntington disease protein via TNT-like protrusion.The Journal of cell biology · 2019Article
- Stem cell therapy for neurological disorders: A focus on aging.Neurobiology of disease · 2019Review
- Successes and Hurdles in Stem Cells Application and Production for Brain Transplantation.Frontiers in neuroscience · 2019Review
- Outcome of cell suspension allografts in a patient with Huntington's disease.Annals of neurology · 2018Article
- Pluripotent Stem Cells for Retinal Tissue Engineering: Current Status and Future Prospects.Stem cell reviews and reports · 2018Review
- Reprogramming Glia Into Neurons in the Peripheral Auditory System as a Solution for Sensorineural Hearing Loss: Lessons From the Central Nervous System.Frontiers in molecular neuroscience · 2018Review
16 more citing papers are in PubMed but not listed here.
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Authors and funding
12 authors at 6 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The clinical evaluation of neural transplantation as a potential treatment for Huntington's disease (HD) was initiated in an attempt to replace lost neurons and improve patient outcomes. Two of 3 patients with HD reported here, who underwent neural transplantation containing striatal anlagen in the striatum a decade earlier, have demonstrated marginal and transient clinical benefits. Their brains were evaluated immunohistochemically and with electron microscopy for markers of projection neurons and interneurons, inflammatory cells, abnormal huntingtin protein, and host-derived connectivity. Surviving grafts were identified bilaterally in 2 of the subjects and displayed classic striatal projection neurons and interneurons. Genetic markers of HD were not expressed within the graft. Here we report in patients with HD that (i) graft survival is attenuated long-term; (ii) grafts undergo disease-like neuronal degeneration with a preferential loss of projection neurons in comparison to interneurons; (iii) immunologically unrelated cells degenerate more rapidly than the patient's neurons, particularly the projection neuron subtype; (iv) graft survival is attenuated in the caudate in comparison to the putamen in HD; (v) glutamatergic cortical neurons project to transplanted striatal neurons; and (vi) microglial inflammatory changes in the grafts specifically target the neuronal components of the grafts. These results, when combined, raise uncertainty about this potential therapeutic approach for the treatment of HD. However, these observations provide new opportunities to investigate the underlying mechanisms involved in HD, as well as to explore additional therapeutic paradigms.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.