Protein disulfide isomerase in ALS mouse glia links protein misfolding with NADPH oxidase-catalyzed superoxide production

Protein disulfide isomerase (PDI) is an oxidoreductase assisting oxidative protein folding in the endoplasmic reticulum of all types of cells, including neurons and glia. In neurodegenerative disorders, such as amyotrophic lateral sclerosis (ALS), up-regulation of PDI is an important part of unfolde...

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Veröffentlicht in:	Human molecular genetics 2013-02, Vol.22 (4), p.646-655
Hauptverfasser:	Jaronen, Merja, Vehviläinen, Piia, Malm, Tarja, Keksa-Goldsteine, Velta, Pollari, Eveliina, Valonen, Piia, Koistinaho, Jari, Goldsteins, Gundars
Format:	Artikel
Sprache:	eng
Schlagworte:	Amyotrophic Lateral Sclerosis - enzymology Amyotrophic Lateral Sclerosis - pathology Animals Anterior Horn Cells - enzymology Astrocytes - enzymology Cell Line Enzyme Activation Enzyme Induction Glial Fibrillary Acidic Protein - metabolism Humans Leukocyte Common Antigens - metabolism Male Mice Mice, Inbred C57BL Mice, Transgenic Microglia - enzymology Microglia - secretion Muscimol - analogs & derivatives Muscimol - pharmacology NADH, NADPH Oxidoreductases - metabolism NADPH Oxidase 1 Procollagen-Proline Dioxygenase - antagonists & inhibitors Procollagen-Proline Dioxygenase - genetics Procollagen-Proline Dioxygenase - metabolism Protein Disulfide-Isomerases - antagonists & inhibitors Protein Disulfide-Isomerases - genetics Protein Disulfide-Isomerases - metabolism Protein Transport Superoxide Dismutase Superoxide Dismutase-1 Superoxides - metabolism Tumor Necrosis Factor-alpha - secretion Unfolded Protein Response
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