Search Results - "Kaufman, R.J"

LMAN1 is a molecular chaperone for the secretion of coagulation factor VIII by Cunningham, M A, Pipe, S W, Zhang, B, Hauri, H-P, Ginsburg, D, Kaufman, R J

“…Combined deficiency of both coagulation factors (F)V and VIII is a rare autosomal recessive bleeding disorder caused by null expression of LMAN1 (previously…”
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Mild Hemophilia A Caused by Increased Rate of Factor VIII A2 Subunit Dissociation: Evidence for Nonproteolytic Inactivation of Factor VIIIa In Vivo by Pipe, S.W., Eickhorst, A.N., McKinley, S.H., Saenko, E.L., Kaufman, R.J.

“…Approximately 5% of hemophilia A patients have normal amounts of a dysfunctional factor VIII (FVIII) protein and are termed cross-reacting material…”
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Mild hemophilia A caused by increased rate of factor VIII A2 subunit dissociation : Evidence for nonproteolytic inactivation of factor VIIIa in vivo by PIPE, S. W, EICKHORST, A. N, MCKINLEY, S. H, SAENKO, E. L, KAUFMAN, R. J

“…Approximately 5% of hemophilia A patients have normal amounts of a dysfunctional factor VIII (FVIII) protein and are termed cross-reacting material…”
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Overexpression of GRP78 mitigates stress induction of glucose regulated proteins and blocks secretion of selective proteins in Chinese hamster ovary cells by Dorner, A.J., Wasley, L.C., Kaufman, R.J.

“…GRP78 is a resident protein of the endoplasmic reticulum (ER) and a member of the glucose regulated protein (GRP) family. Many secretion incompetent proteins…”
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Post-translational requirements for functional factor V and factor VIII secretion in mammalian cells by PITTMAN, D. D, TOMKINSON, K. N, KAUFMAN, R. J

“…Coagulation factors V and VIII are homologous glycosylated plasma proteins that provide essential functions for hemostasis. Factor V is secreted as a single…”
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Increased synthesis of secreted proteins induces expression of glucose-regulated proteins in butyrate-treated Chinese hamster ovary cells by Dorner, A.J., Wasley, L.C., Kaufman, R.J.

“…The effects of increased synthesis of secreted proteins expressed from stably integrated heterologous genes in Chinese hamster ovary (CHO) cells following…”
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PACE/furin can process the vitamin K-dependent pro-factor IX precursor within the secretory pathway by WASLEY, L. C, REHEMTULLA, A, BRISTOL, J. A, KAUFMAN, R. J

“…Factor IX is synthesized as a precursor polypeptide which requires proteolytic cleavage of the propeptide for functional activity. Expression of factor IX at…”
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Protein folding, oxidative stress, and the unfolded protein response by Kaufman, R.J.

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unfolded protein response is required to maintain the integrity of the endoplasmic reticulum, prevent oxidative stress and preserve differentiation in β-cells by Kaufman, R.J, Back, S.H, Song, B, Han, J, Hassler, J

“…Diabetes is an epidemic of worldwide proportions caused by β-cell failure. Nutrient fluctuations and insulin resistance drive β-cells to synthesize insulin…”
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Adaptive Preconditioning in Neurological Diseases – Therapeutic Insights from Proteostatic Perturbations by Mollereau, B, Rzechorzek, NM, Roussel, BD, Sedru, M, Van den Brink, D, Bailly-Maitre, B, Palladino, F, Medinas, D.B, Domingos, PM, Hunot, S, Chandran, S, Birman, S, Baron, T, Vivien, D, Duarte, CB, Ryoo, HD, Steller, H, Urano, F, Chevet, E, Kroemer, G, Ciechanover, A, Calabrese, EJ, Kaufman, RJ, Hetz, C

“…Abstract In neurological disorders, both acute and chronic neural stress can disrupt cellular proteostasis, resulting in the generation of pathological…”
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Protein folding in the endoplasmic reticulum and the unfolded protein response by Zhang, K, Kaufman, R J

“…In all eukaryotic cells, the endoplasmic reticulum (ER) is an intracellular organelle where folding and assembly occurs for proteins destined to the…”
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Cantharidins Induce ER Stress and a Terminal Unfolded Protein Response in OSCC by Xi, Y., Garshott, D.M., Brownell, A.L., Yoo, G.H., Lin, H.-S., Freeburg, T.L., Yoo, N.G., Kaufman, R.J., Callaghan, M.U., Fribley, A.M.

“…Mortality and morbidity associated with oral squamous cell carcinoma (OSCC) remain unacceptably high with disfiguring treatment options and a death rate of 1…”
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Bioengineering of coagulation factor VIII for efficient expression through elimination of a dispensable disulfide loop by SELVARAJ, S. R., SCHELLER, A. N., MIAO, H. Z., KAUFMAN, R. J., PIPE, S. W.

“…Background: Heterologous expression of factor VIII (FVIII) is about two to three orders of magnitude lower than similarly sized proteins. Bioengineering…”
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ANTI-OXIDANTS IMPROVE FACTOR VIII FOLDING AND SECRETION AND REDUCE CELL TOXICITY AND INFLAMMATION IN VIVO IN MICE by Kaufman, R.J., Malhotra, J., Pipe, S.W., Miao, H.

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unfolded protein response represses differentiation through the RPD3-SIN3 histone deacetylase by Schroder, M, Clark, R, Liu, C.Y, Kaufman, R.J

“…In Saccharomyces cerevisiae, splicing of HAC1 mRNA is initiated in response to the accumulation of unfolded proteins in the endoplasmic reticulum by the…”
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EFFICIENT SECRETION OF BIOENGINEERED COAGULATION FACTOR VIII INTO THE MILK OF TRANSGENIC ANIMALS by Pipe, S.W., Miao, H.Z., Butler, S.T., Kaufman, R.J., Calcaterra, J., Velander, W.H.

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Effects of N-linked carbohydrate on the in vivo properties of human GM-CSF by Donahue, R E, Wang, E A, Kaufman, R J, Foutch, L, Leary, A C, Witek-Giannetti, J S, Metzger, M, Hewick, R M, Steinbrink, D R, Shaw, G

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The unfolded protein response pathway in Saccharomyces cerevisiae. Oligomerization and trans-phosphorylation of Ire1p (Ern1p) are required for kinase activation by Welihinda, A.A. (University of Michigan Medical Center, Ann Arbor.), Kaufman, R.J

“…In eukaryotic cells, accumulation of unfolded proteins in the endoplasmic reticulum (ER) results in a transcription induction of a number of ER chaperone…”
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The Transcriptional Co-activator ADA5 Is Required for HAC1 mRNA Processing in Vivo by Welihinda, Ajith A., Tirasophon, Witoon, Kaufman, Randal J.

“…Accumulation of unfolded proteins in the endoplasmic reticulum (ER) activates signaling pathways to induce transcription of a number of genes encoding ER…”
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Good things come in small packages for hemophilia by Kaufman, R. J.

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