Publications by authors named "G Fruhmann"

FENIB (familial encephalopathy with neuroserpin inclusion bodies) is a human monogenic disease caused by point mutations in the gene, characterized by the intracellular deposition of polymers of neuroserpin (NS), which leads to proteotoxicity and cell death. Despite the different cell and animal models developed thus far, the exact mechanism of cell toxicity elicited by NS polymers remains unclear. Here, we report that human wild-type NS and the polymerogenic variant G392E NS form protein aggregates mainly localized within the endoplasmic reticulum (ER) when expressed in the yeast .

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The photo differential scanning calorimetry (photo-DSC) is an appropriate method to characterize photopolymers used in additive manufacturing (AM). Important process parameters such as optimal ultraviolet (UV) exposure time and reaction heat can be attained by this method. However, achieving reliable and meaningful results from photo-DSC experiments requires careful sample preparation, i.

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Aβ metabolism plays a pivotal role in Alzheimer's disease. Here, we used a yeast model to monitor Aβ toxicity when entering the secretory pathway and demonstrate that processing in, and exit from the endoplasmic reticulum (ER) is required to unleash the full Aβ toxic potential. Consistent with previously reported data, our data suggests that Aβ interacts with mitochondria, thereby enhancing formation of reactive oxygen species and eventually leading to cell demise.

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In this review article, yeast model-based research advances regarding the role of Amyloid-β (Aβ), Tau and frameshift Ubiquitin UBB in Alzheimer’s disease (AD) are discussed. Despite having limitations with regard to intercellular and cognitive AD aspects, these models have clearly shown their added value as complementary models for the study of the molecular aspects of these proteins, including their interplay with AD-related cellular processes such as mitochondrial dysfunction and altered proteostasis. Moreover, these yeast models have also shown their importance in translational research, e.

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Neurodegenerative disorders have a profound effect on the quality of life of patients and their environment. However, the development of adequate therapies requires accurate understanding of the underlying disease pathogenesis. On that account, yeast models can play an important role, as they enable the elucidation of the mechanisms leading to neurodegenerative disorders.

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