Global ETD Search
Search theses and dissertations gathered from participating repositories worldwide. Every result links back to the library that holds it. No account is needed.
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Showing 1 to 20 of 23 for “"SCA1"”.
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The role of gene expression and aging in SCA1
Spinocerebellar ataxia type 1 is an autosomal dominant disorder caused by a CAG repeat expansion encoding a polyglutamine tract, where patients present with a lack of motor coordination including ataxia. The disease is characterized pathologically by loss of Purkinje cells (PCs) in the cerebellar …
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Identification of a suitable SNP for allele-specific silencing of the disease-causing gene in SCA1 patients in South Africa
Spinocerebellar ataxia 1 (SCA1) is part of a broader group of dominant neurodegenerative disorders caused by an unstable CAG trinucleotide repeat. There is no known cure for this disease and symptoms worsen progressively culminating in death. The disease-causing mutation in SCA1 occurs in the ATXN1 …
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A genome-wide, single-cell analysis of vascular smooth muscle cell plasticity
… subset of VSMCs expressing Stem cell antigen 1 (SCA1). Single-cell RNA-seq was combined with VSMC-specific lineage tracing to profile gene expression in individual VSMCs from healthy mouse arteries and to compare SCA1-expressing VSMCs to other cells. SCA1-positive VSMCs were heterogeneous, with …
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Intra-Regional Differences in Cerebellar Vulnerability of Spinocerebellar Ataxia Type 1 Mice
… an open question. Spinocerebellar Ataxia type 1 (SCA1) is a dominantly inherited neurodegenerative disease caused by an abnormal expansion of polyglutamine (polyQ) repeats in the ATAXIN1 (ATXN1) gene and characterized by cerebellar degeneration. Recent studies in patients with SCA1 indicate that …
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The Role of Microglia and Astrocyte in Spinocerebellar Ataxia Type 1
Spinocerebellar ataxia type 1 (SCA1) is a fatal dominantly inherited neurodegenerative disease. Even though there has been illuminating work on the effect of the disease-causing protein, a polyQ expanded ATAXIN-1 (ATXN1) on neurons, the relative contribution to disease of glia has been unknown. …
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Magnetresonanztomographie-basierte Volumetrie bei hereditären spinozerebellären Ataxien
… zu quantifizieren, wurde bei 82 Patienten mit SCA1, SCA3 oder SCA6 sowie bei 31 Kontrollpersonen nach vorangehender Bildgebung mittels Magnetresonanztomographie (MRT) eine anschließende quantitative 3D-Volumetrie durchgeführt. Diese Untersuchung ergab für SCA1 und SCA3 schwere Atrophien des …
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Heterogeneity in vascular smooth muscle cell gene expression and its association with clonal proliferation in models of vascular disease
… identified VSMCs expressing stem cell antigen-1 (Sca1), which lacked expression of conventional VSMC markers, but were also distinct from adventitial and endothelial cell profiles. Induction of Sca1 was evident in phenotypically switched VSMCs in vitro and in both in vivo disease models. Notably, …
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The mesenchymal regulation of ductal-driven liver regeneration
… to 20% of the cells by the stem cell antigen 1 (SCA1). In vivo, SCA1+PDGFRα+ mesenchymal cells localise periportally, closely surrounding biliary duct cells, and co-expanding with them during damage- induced regeneration. Isolated SCA1+ mesenchymal cells express key pro- regenerative factors …
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Ataxin-1 in cognition and mood
… gene mutated in spinocerebellar ataxia type 1 (SCA1), may affect cognition and mood, but much remains unknown, including which brain areas are responsible, whether ATXN1 affects mood in mice, and the mechanisms of these effects. To answer these questions, we characterized cognition and mood in …
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Defining a Neuroprotective Pathway for the Treatment of Ataxias
… of the cerebellum. Mouse models of SCA Type 1 (SCA1) can be used to study the molecular mechanisms underlying PC degeneration and death. One SCA1 mouse model, ATXN1[30Q]D776, has an initial ataxia but no progressive degeneration or PC death. RNA-seq experiments identified the up-regulation in …
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An alternative B cell development program
… unclear. We showed that FO II cells express more Sca1 than FO I cells and originate from a distinct B cell development program, marked by high expression of Sca1. MZ B cells can derive from the “canonical” Sca1lo pathways, as well as from the Sca1hi program, although the Sca1hi program shows a …
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Investigating the Mechanisms Underlying the Heterogeneous VSMC Contribution to Vascular Disease
… that VSMCs marked by stem cell antigen-1 (SCA1) may represent such a primed population, profiling of chromatin accessibility in SCA1+ VSMCs revealed substantial opening of chromatin at genes showing increased expression in injury-activated compared to healthy VSMCs. Manipulation of RUNX1 …
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Development and Maintenance of Thymic Epithelial Microenvironment
… expressed the surface makers PDGFRα, PDGFRβ, SCA1, CD29, CD44, CD49f, and CD90 identical to markers Used to characterize mesenchymal stem cells. LRC were isolated and maintained As a thymic mesenchymal stem cell line (TMSC), which showed a limited Capability for forming a reaggregated thymus …
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Downregulation of Prdm16 Is Critical for HOXB4-mediated Benign HSC Expansion In Vivo
… changes at multiple time points in Lin-Sca1+c-kit+ (LSK) cells from mice transplanted with bone marrow (BM) cells transduced with a MSCV-HOXB4-ires-YFP vector. A distinct HOXB4 transcriptional program was reproducibly induced and stabilized by 12 weeks after transplant. Dynamic …
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Cellular plasticity in white adipose tissue: in vivo identification of bipotent adipocyte progenitors in adult white adipose tissue
… growth factor receptor alpha (PDGFRA), CD34 and Sca1 (PDGFRA+ cells). PDGFRA+ cells have a unique morphology in which extended processes contact multiple cells in the tissue microenvironment. Surprisingly, these cells also give rise to white adipocytes (WA) that can comprise up to 25% of total …
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Identification and characterisation of a novel, multi-potent, skeletal muscle-derived stem cell with broad developmental plasticity
… assessed in cardiac tissue, and in an isolated Sca1+ cardiac stem cell population. Satellite cells and PICs were identified and quantified in hind limb skeletal muscle of 3, 10 and 21 day, and 2 year old mice: there was a decline in abundance of both SC and PICs with age. PICs were isolated by …
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Hematopoietic Stem Cells and Progenitors in Murine Autoimmune Arthritis
… Gene expression analysis of uncommitted Kit+Sca1+Lin-: KSL) cells from arthritic bone marrow revealed increased expression of myeloid cell related transcripts at the expense of megakaryocyte and erythroid transcripts. In vitro, KSL cells from arthritic mice were markedly superior in …
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A Role for PI31-Mediated Proteasome Regulation in Proteostasis and Neuronal Health
… model of spinocerebellar ataxia type 1(SCA1). These results demonstrate a link between NAD+ and proteasomes that may ultimately prove useful for developing interventions that counter the effects of neurodegeneration and allow for an understanding why this system begins to fail in aging …
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THE ROLE OF SDF-1[alpha] AS A VASCULOGENIC CHEMOKINE AND ENDOTHELIUM-ASSOCIATED CELL ADHESION MOLECULE FOR THE RECRUITMENT OF BONE MARROW-DERIVED PROGENITOR CELLS TO DEVELOPING TUMORS
… tumors (DTC). Lineage-negative BMC enriched for Sca1+cKit+ (LSK) cells, including endothelial progenitors and CXCR4+ cells, were used for subsequent adhesion studies. LSK cells preferentially adhered in vitro to DTC, compared to other target cell types. Pre-incubation with anti-CXCR4 antibody or …
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Identification of a vascular smooth muscle cell transition associated with fibrous cap formation and induced by thrombin receptor activation
… intermediate VSMC population marked by Ly6a/SCA1, Vcam1 and Lgals3 expression – a population that I term “intermediate modulated VSMCs” (imVSMCs). Multi-colour VSMC lineage tracing and immunostaining analyses indicated that NOTCH3+ fcVSMCs and VCAM1+ imVSMCs were present in the same clonal …
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