Search Results - "Perri, Emma R."

ERp57 is protective against mutant SOD1-induced cellular pathology in amyotrophic lateral sclerosis by Parakh, Sonam, Jagaraj, Cyril J, Vidal, Marta, Ragagnin, Audrey M G, Perri, Emma R, Konopka, Anna, Toth, Reka P, Galper, Jasmin, Blair, Ian P, Thomas, Colleen J, Walker, Adam K, Yang, Shu, Spencer, Damian M, Atkin, Julie D

“…Abstract Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder and mutations in superoxide dismutase 1 (SOD1) account for 20% of familial…”
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Protein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia Continuum by Shahheydari, Hamideh, Ragagnin, Audrey, Walker, Adam K, Toth, Reka P, Vidal, Marta, Jagaraj, Cyril J, Perri, Emma R, Konopka, Anna, Sultana, Jessica M, Atkin, Julie D

“…Protein homeostasis, or proteostasis, has an important regulatory role in cellular function. Protein quality control mechanisms, including protein folding and…”
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The Redox Activity of Protein Disulfide Isomerase Inhibits ALS Phenotypes in Cellular and Zebrafish Models by Parakh, Sonam, Shadfar, Sina, Perri, Emma R., Ragagnin, Audrey M.G., Piattoni, Claudia V., Fogolín, Mariela B., Yuan, Kristy C., Shahheydari, Hamideh, Don, Emily K., Thomas, Collen J., Hong, Yuning, Comini, Marcelo A., Laird, Angela S., Spencer, Damian M., Atkin, Julie D.

“…Pathological forms of TAR DNA-binding protein 43 (TDP-43) are present in almost all cases of amyotrophic lateral sclerosis (ALS), and 20% of familial ALS cases…”
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The Unfolded Protein Response and the Role of Protein Disulfide Isomerase in Neurodegeneration by Perri, Emma R, Thomas, Colleen J, Parakh, Sonam, Spencer, Damian M, Atkin, Julie D

“…The maintenance and regulation of proteostasis is a critical function for post-mitotic neurons and its dysregulation is increasingly implicated in…”
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Protein Disulfide Isomerase Endoplasmic Reticulum Protein 57 (ERp57) is Protective Against ALS-Associated Mutant TDP-43 in Neuronal Cells by Parakh, Sonam, Perri, Emma R., Vidal, Marta, Takalloo, Zeinab, Jagaraj, Cyril J., Mehta, Prachi, Yang, Shu, Thomas, Colleen J., Blair, Ian P., Hong, Yuning, Atkin, Julie D.

“…Amyotrophic Lateral Sclerosis (ALS) is a severe neurodegenerative disease affecting motor neurons. Pathological forms of Tar-DNA binding protein-43 (TDP-43),…”
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The Cysteine (Cys) Residues Cys-6 and Cys-111 in Mutant Superoxide Dismutase 1 (SOD1) A4V Are Required for Induction of Endoplasmic Reticulum Stress in Amyotrophic Lateral Sclerosis by Perri, Emma R., Parakh, Sonam, Vidal, Marta, Mehta, Prachi, Ma, Yi, Walker, Adam K., Atkin, Julie D.

“…Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the degeneration of motor neurons. Between 12 and 20% of inherited cases…”
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Correction to: the Cysteine (Cys) Residues Cys-6 and Cys-111 in Mutant Superoxide Dismutase 1 (SOD1) A4V Are Required for Induction of Endoplasmic Reticulum Stress in Amyotrophic Lateral Sclerosis by Perri, Emma R., Parakh, Sonam, Vidal, Marta, Mehta, Prachi, Ma, Yi, Walker, Adam K., Atkin, Julie D.

“…The original version of this article unfortunately contained an error in Fig. 3. The image shown for “C57S” was incorrect…”
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