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Many trinucleotide repeat disorders exhibit region-specific toxicity within tissues, the basis of which cannot be explained by traditional methods. For example, in Huntington’s Disease (HD), the toxic disease-causing protein is ubiquitously expressed. However, only the medium ...

Ataxin-1 protein expression is found in the cytoplasm and nucleus of Purkinje cells, the primary site of spinocerebellar ataxia type 1 (SCA1). Phosphorylation at S776 occurs in the cytoplasm and stabilizes the protein through interaction with 14-3-3, allowing it to translocate into the nu ...

Huntington’s disease (HD) is a hereditary neurodegenerative disorder, characterized by motor, psychiatric, and cognitive symptoms. The genetic defect responsible for the onset of the disease, expansion of CAG repeats in exon 1 of the gene that codes for huntingtin, has been unambiguous ...

The fragile X syndrome (FXS), the most common cause of heritable intellectual disability, is caused by expansion of a CGG repeat located at the 5′ UTR of the FMR1 gene and subsequent epigenetic modifications of its promoter. Epigenetic modifications include both methylation of the cytosines ...

Combining multicolor fluorescent in situ hybridization (FISH) and immunofluorescent staining (IFS) presents a powerful method for visualizing the spatial relationship between mRNA and proteins in different neural compartments. Although seemingly straightforward, t ...

Fragile X syndrome results from the lack of FMR1 expression. To understand how the lack of FMR1 function leads to the syndrome, we are studying the Drosophila FMR1 related protein (dFMR1). We performed affinity purification of dFMR1-associated complexes from cultured Drosophila S2 cells ...

This chapter describes the potential use of viral-mediated gene transfer in the central nervous system for the silencing of gene expression using RNA interference in the context of Huntington’s disease (HD). Protocols provided here describe the design of small interfering RNAs, their e ...

Mesenchymal stem cells/marrow stromal cells (MSCs) are ideally suited for cellular therapy due to their ease of isolation, manipulation, and strong safety profile in the clinic. They can be expanded from normal qualified human donors in large quantities and can be infused without tissue ma ...

Huntington’s disease (HD) is a devastating disease that currently has no cure. Transgenic HD monkeys have developed key neuropathological and cognitive behavioral impairments similar to HD patients. Thus, pluripotent stem cells derived from transgenic HD monkeys could be a useful c ...

The mammalian motor cortex is capable of circuit reorganization driven by acquisition of novel motor skills. Time-lapse imaging of synaptic structures in the living brain provides valuable information on how motor learning rewires synaptic connections and how long-lasting memo ...

Transcranial two-photon microscopy allows long-term imaging of neurons, glia, and vasculature in the intact cortex of living animals. So far, this technique has been primarily used to acquire images in anesthetized animals. Here, we describe a detailed protocol for high-resolution two ...

Huntington’s disease (HD) is an inherited neurodegenerative disease that is characterized by movement abnormalities, cognitive impairment, and abnormal behavior as well as sleep and weight problems. It is an autosomal dominant disorder caused by a mutation in the huntingtin gene on t ...

Instability of repetitive DNA sequences within the genome is associated with a number of human diseases. The expansion of trinucleotide repeats is recognized as a major cause of neurological and neuromuscular diseases, and progress in understanding the mutations over the last 20 years h ...

Investigation of cardiac progenitor cell proliferation and differentiation is essential for both the basic understanding of progenitor cell biology as well as the development of cellular therapeutics for tissue regeneration. Herein, we describe techniques used for the analys ...

The ability to identify the donor stem cells following transplantation into injured hearts is critical. This is particularly important in evaluating stem cell survival and lineage differentiation into mature cardiovascular cells. Several approaches have been employed for tra ...

Human-induced pluripotent stem cells (hiPSCs) have received enormous attention because of their ability to differentiate into multiple cell types that demonstrate the patient’s original phenotype. The use of hiPSCs is particularly valuable to the study of cardiac biology, as human ...

Cardiac progenitor cells (CPC) are a unique pool of progenitor cells residing in the heart that play an important role in cardiac homeostasis and physiological cardiovascular cell turnover during acute myocardial infarction (MI). Transplanting CPC into the heart has shown promise in t ...

Since the resident cardiac stem/progenitor cells were discovered, their ability to maintain the architecture and functional integrity of adult heart has been broadly explored. The methods for isolation and purification of the cardiac stem cells are crucial for the precise analysis of ...

Over the last decade, cell therapy has emerged as a potentially new approach for the treatment of cardiovascular diseases. Among the wide range of cell types and sources, adipose-derived mesenchymal stem cells have shown promise, mainly due to its plasticity and remarkable paracrine-sec ...

The “mesenchymal stem cells (MSCs)” are cells adherent in the bone marrow, which can be isolated to induce differentiation. In contrast to the “embryonic stem cells” whose goal is to develop a new organism, the “MSC adult stem cells” can participate in tissue growth and repair throughout postnatal ...