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 9 of 9 for “"lysosomal proteins"”.
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The Role of Progranulin in Frontotemporal Dementia
… mass spectrometry to define robust lysosomal proteomic aberrations in progranulin haploinsufficient cells in both Grn heterozygous mouse models as well as GRN-FTD patient derived fibroblasts. This led to the discovery that progranulin haploinsufficiency leads to decreases in dozens …
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Cytosolic sphingolipids and lysosome reactivation in neurodegenerative diseases
… an activator of vATPase, leading to proper endolysosomal acidification. Lysosomal storage disorders (LSDs), are caused by the defective activity of lysosomal proteins, including the accumulation of unmetabolized substrates. The accumulation of substrates is thought to initiate a complex …
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Biochemical Characterization of Niemann-Pick C: A Disease of Cholesterol Transport
… where cholesterol is liberated from plasma lipoproteins that have entered the cell through receptor-mediated endocytosis. This cholesterol is transported from the lysosome to other cellular membranes to perform structural and regulatory roles. A clue to the mechanism of this cholesterol movement …
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Identification of Cathepsin B and L as Novel Uva Targets Upstream of Cutaneous Lysosomal-Autophagic Dysregulation
… inactivation upstream of autophagic-lysosomal dysfunction. In human skin fibroblasts, exposure to noncytotoxic doses of chronic UVA (9.9 J/cm ², twice a week, 3 weeks) caused pronounced photooxidative impairment of cathepsin B and L enzymatic activity suppressed by antioxidant …
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Using high-throughput proteomics to identify novel engineering targets for enhanced rAAV production in HEK293 cells
… was determined by quantifying HEK293-VP 2.0 cell proteins in mock transfected and rAAV2-, rAAV6.2- and rAAV8-producing cells at 48- and 96-hours post-transfection. A total of 7,469 and 7,180 cellular proteins were quantified by TMT shotgun proteomics at each timepoint, respectively. Next, the …
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Phenotypic characterisation of human iPSC neuronal models of GM2 gangliosidoses
… the effects of their accumulation in ganglioside lysosomal storage disorders (LSD). The GM2 gangliosidoses Tay-Sachs and Sandhoff disease are a type of LSD, resulting from the inability of the lysosome to catabolise the breakdown of the ganglioside GM2. This is due to a loss or mutation of either …
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The functional role of the retromer complex in the pathogenesis of Alzheimer's disease in Down syndrome
… patients with DS. Retromer recognition core proteins were significantly decreased in both the hippocampi and cortices of young and aged DS subjects compared to controls. Correlative analyses showed a significant inverse relationship between recognition core proteins and levels of soluble …
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Modalities of Cholesterol Binding and Modulation of the NPC Proteins and Scap
Low density lipoproteins (LDL) and related plasma lipoproteins deliver cholesterol to cells by receptor-mediated endocytosis. The lipoprotein is degraded in late endosomes and lysosomes, allowing cholesterol to be released. Export of cholesterol from late endosomes and lysosomes (hereafter referred …
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Identifizierung und molekulare Charakterisierung des lysosomalen Matrixproteins Serincarboxypeptidase 1
Die lysosomale Matrix enthält mehr als 50 hydrolytische Enzyme, die an der Degradation von Makromolekülen wie Polysacchariden, Proteinen, Nukleinsäuren und Lipiden beteiligt sind. Neben ihrer klassischen katabolischen Funktion sind die Lysosomen zudem an einer Vielzahl von spezialisierten, …