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    Results: 1 to 20 of 231

    1.

    Dominant negative pathogenesis by mutant proinsulin in the Akita diabetic mouse.

    Izumi T, Yokota-Hashimoto H, Zhao S, Wang J, Halban PA, Takeuchi T.

    Diabetes. 2003 Feb;52(2):409-16.PMID: 12540615 [PubMed - indexed for MEDLINE]Related articlesFree article

    2.

    A mutation in the insulin 2 gene induces diabetes with severe pancreatic beta-cell dysfunction in the Mody mouse.

    Wang J, Takeuchi T, Tanaka S, Kubo SK, Kayo T, Lu D, Takata K, Koizumi A, Izumi T.

    J Clin Invest. 1999 Jan;103(1):27-37.PMID: 9884331 [PubMed - indexed for MEDLINE]Related articlesFree article

    3.

    Misfolded proinsulin accumulates in expanded pre-Golgi intermediates and endoplasmic reticulum subdomains in pancreatic beta cells of Akita mice.

    Zuber C, Fan JY, Guhl B, Roth J.

    FASEB J. 2004 May;18(7):917-9. Epub 2004 Mar 19.PMID: 15033933 [PubMed - indexed for MEDLINE]Related articlesFree article

    4.

    Expression of mutant Ins2C96Y results in enhanced tubule formation causing enlargement of pre-Golgi intermediates of CHO cells.

    Fan JY, Roth J, Zuber C.

    Histochem Cell Biol. 2007 Aug;128(2):161-73. Epub 2007 Jul 24.PMID: 17647009 [PubMed - indexed for MEDLINE]Related articles

    5.

    Proinsulin disulfide maturation and misfolding in the endoplasmic reticulum.

    Liu M, Li Y, Cavener D, Arvan P.

    J Biol Chem. 2005 Apr 8;280(14):13209-12. Epub 2005 Feb 10.PMID: 15705595 [PubMed - indexed for MEDLINE]Related articlesFree article

    6.

    Proinsulin maturation, misfolding, and proteotoxicity.

    Liu M, Hodish I, Rhodes CJ, Arvan P.

    Proc Natl Acad Sci U S A. 2007 Oct 2;104(40):15841-6. Epub 2007 Sep 26.PMID: 17898179 [PubMed - indexed for MEDLINE]Related articlesFree article

    7.

    Impact of endoplasmic reticulum stress pathway on pancreatic beta-cells and diabetes mellitus.

    Araki E, Oyadomari S, Mori M.

    Exp Biol Med (Maywood). 2003 Nov;228(10):1213-7. Review.PMID: 14610263 [PubMed - indexed for MEDLINE]Related articlesFree article

    8.

    Type 1 von Willebrand disease mutation Cys1149Arg causes intracellular retention and degradation of heterodimers: a possible general mechanism for dominant mutations of oligomeric proteins.

    Bodó I, Katsumi A, Tuley EA, Eikenboom JC, Dong Z, Sadler JE.

    Blood. 2001 Nov 15;98(10):2973-9.PMID: 11698279 [PubMed - indexed for MEDLINE]Related articlesFree article

    9.

    Proinsulin lacking the A7-B7 disulfide bond, Ins2Akita, tends to aggregate due to the exposed hydrophobic surface.

    Yoshinaga T, Nakatome K, Nozaki J, Naitoh M, Hoseki J, Kubota H, Nagata K, Koizumi A.

    Biol Chem. 2005 Nov;386(11):1077-85.PMID: 16307473 [PubMed - indexed for MEDLINE]Related articles

    10.

    Identification and characterization of two novel mutations (Q421 K and R123P) in congenital factor XII deficiency.

    Kanaji T, Kanaji S, Osaki K, Kuroiwa M, Sakaguchi M, Mihara K, Niho Y, Okamura T.

    Thromb Haemost. 2001 Dec;86(6):1409-15.PMID: 11776307 [PubMed - indexed for MEDLINE]Related articles

    11.

    Proteotoxicity in the endoplasmic reticulum: lessons from the Akita diabetic mouse.

    Ron D.

    J Clin Invest. 2002 Feb;109(4):443-5. Review. No abstract available. PMID: 11854314 [PubMed - indexed for MEDLINE]Related articlesFree article

    12.

    A mutant human proinsulin is secreted from islets of Langerhans in increased amounts via an unregulated pathway.

    Carroll RJ, Hammer RE, Chan SJ, Swift HH, Rubenstein AH, Steiner DF.

    Proc Natl Acad Sci U S A. 1988 Dec;85(23):8943-7.PMID: 3057496 [PubMed - indexed for MEDLINE]Related articlesFree article

    13.

    Mutant PrP is delayed in its exit from the endoplasmic reticulum, but neither wild-type nor mutant PrP undergoes retrotranslocation prior to proteasomal degradation.

    Drisaldi B, Stewart RS, Adles C, Stewart LR, Quaglio E, Biasini E, Fioriti L, Chiesa R, Harris DA.

    J Biol Chem. 2003 Jun 13;278(24):21732-43. Epub 2003 Mar 26.PMID: 12663673 [PubMed - indexed for MEDLINE]Related articlesFree article

    14.

    Intracellular transport of proinsulin in pancreatic beta-cells. Structural maturation probed by disulfide accessibility.

    Huang XF, Arvan P.

    J Biol Chem. 1995 Sep 1;270(35):20417-23.PMID: 7657617 [PubMed - indexed for MEDLINE]Related articlesFree article

    15.

    Hyperproinsulinemia in a three-generation Caucasian family due to mutant proinsulin (Arg65-His) not associated with imparied glucose tolerance: the contribution of mutant proinsulin to insulin bioactivity.

    Röder ME, Vissing H, Nauck MA.

    J Clin Endocrinol Metab. 1996 Apr;81(4):1634-40. Review.PMID: 8636380 [PubMed - indexed for MEDLINE]Related articles

    16.

    Intracellular degradation of secretion defect-type mutants of antithrombin is inhibited by proteasomal inhibitors.

    Tokunaga F, Shirotani H, Hara K, Kozuki D, Omura S, Koide T.

    FEBS Lett. 1997 Jul 21;412(1):65-9. Erratum in: FEBS Lett 1997 Oct 6;415(3):351. PMID: 9257691 [PubMed - indexed for MEDLINE]Related articles

    17.

    Control of mRNA translation preserves endoplasmic reticulum function in beta cells and maintains glucose homeostasis.

    Scheuner D, Vander Mierde D, Song B, Flamez D, Creemers JW, Tsukamoto K, Ribick M, Schuit FC, Kaufman RJ.

    Nat Med. 2005 Jul;11(7):757-64. Epub 2005 Jun 26.PMID: 15980866 [PubMed - indexed for MEDLINE]Related articles

    18.

    The endoplasmic reticulum stress response is stimulated through the continuous activation of transcription factors ATF6 and XBP1 in Ins2+/Akita pancreatic beta cells.

    Nozaki J, Kubota H, Yoshida H, Naitoh M, Goji J, Yoshinaga T, Mori K, Koizumi A, Nagata K.

    Genes Cells. 2004 Mar;9(3):261-70.PMID: 15005713 [PubMed - indexed for MEDLINE]Related articles

    19.

    Mutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulum.

    Miranda E, Römisch K, Lomas DA.

    J Biol Chem. 2004 Jul 2;279(27):28283-91. Epub 2004 Apr 16.PMID: 15090543 [PubMed - indexed for MEDLINE]Related articlesFree article

    20.

    Role of SREBP-1 in the development of parasympathetic dysfunction in the hearts of type 1 diabetic Akita mice.

    Park HJ, Zhang Y, Du C, Welzig CM, Madias C, Aronovitz MJ, Georgescu SP, Naggar I, Wang B, Kim YB, Blaustein RO, Karas RH, Liao R, Mathews CE, Galper JB.

    Circ Res. 2009 Jul 31;105(3):287-94. Epub 2009 May 7.PMID: 19423844 [PubMed - indexed for MEDLINE]Related articlesFree article

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