Matthew D. Benson

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The mitochondria-associated membrane (MAM) is a domain of the endoplasmic reticulum (ER) that mediates the exchange of ions, lipids and metabolites between the ER and mitochondria. ER chaperones and oxidoreductases are critical components of the MAM. However, the localization motifs and mechanisms for most MAM proteins have remained elusive. Using two(More)
Protein secretion from the endoplasmic reticulum (ER) requires the enzymatic activity of chaperones and oxidoreductases that fold polypeptides and form disulfide bonds within newly synthesized proteins. The best-characterized ER redox relay depends on the transfer of oxidizing equivalents from molecular oxygen through ER oxidoreductin 1 (Ero1) and protein(More)
The palmitoylation of calnexin serves to enrich calnexin on the mitochondria-associated membrane (MAM). Given a lack of information on the significance of this finding, we have investigated how this endoplasmic reticulum (ER)-internal sorting signal affects the functions of calnexin. Our results demonstrate that palmitoylated calnexin interacts with(More)
The mitochondria-associated membrane (MAM) has emerged as an endoplasmic reticulum (ER) signaling hub that accommodates ER chaperones, including the lectin calnexin. At the MAM, these chaperones control ER homeostasis but also play a role in the onset of ER stress-mediated apoptosis, likely through the modulation of ER calcium signaling. These opposing(More)
Michael Bui, Susanna Y. Gilady, Ross E. B. Fitzsimmons, Matthew D. Benson, Emily M. Lynes, Kevin Gesson, Neal M. Alto, Stefan Strack, John D. Scott , and Thomas Simmen From the Department of Cell Biology, School of Molecular and Systems Medicine, University of Alberta, Edmonton, Alberta T6G 2H7, Canada, the Department of Microbiology, University of Texas(More)
Amyloidosis has received considerable attention recently because of its association with Alzheimer's disease. Actually, the amyloid in the cortical plaques, which is characteristic of Alzheimer's disease, is a localized form of amyloid deposition. Although intracranial vascular amyloid deposits which contain the A4 or beta-protein are usually associated(More)
Die Einteilung der familiären Amyloidneuropathien (FAP) erfolgte nach regionalen und klinischen Aspekten. In den letzten 10 Jahren haben sich mehr als 40 Punktmutationen auf dem Transthyretin (TTR)-Gen als Ursache für die FAP und andere Amyloidosen nachweisen lassen. Die FAP II, ausgelöst durch eine Punktmutation am Codon 58 des TTR-Gens, ist bislang nur(More)
The palmitoylation of calnexin serves to enrich calnexin on the mitochondria-associated membrane (MAM). Given a lack of information on the significance of this finding, we have investigated how this endoplasmic reticulum (ER)-internal sorting signal affects the functions of calnexin. Our results demonstrate that palmitoylated calnexin interacts with 5(More)
PURPOSE To report a rare case of congenital glaucoma in a patient with CHARGE syndrome, present gonioscopic photographs, and explore mechanisms of disease that may account for this association. PATIENTS AND METHODS We describe a 35-week-old girl with previously diagnosed CHARGE syndrome who presented with corneal edema, buphthalmos, and elevated(More)
Received 6 May 1992. Revised version accepted 23 July 1992. Abstract A transthyretin (TTR) mutation is described in a 44 year old French woman from Caen who presented at the age of 40 with neuropathy in all four extremities, diarrhoea, and orthostatic hypotension. Her father died with a similar syndrome including vitreous opacities. A nerve biopsy from the(More)