9 results found
    1. Cell Biology

    Proinsulin misfolding is an early event in the progression to type 2 diabetes

    Anoop Arunagiri et al.
    Proinsulin misfolding, an established cause of diabetes in patients with INS gene mutations, is now observed in normal human pancreatic islets, and rodents with genetic predisposition to type 2 diabetes.
    1. Human Biology and Medicine

    Inhibition of mTORC1 by ER stress impairs neonatal β-cell expansion and predisposes to diabetes in the Akita mouse

    Yael Riahi et al.
    mTORC1 inhibition by perinatal ER stress induces beta-cell growth arrest with subsequent development of diabetes.
    1. Stem Cells and Regenerative Medicine

    Insulin mutations impair beta-cell development in a patient-derived iPSC model of neonatal diabetes

    Diego Balboa et al.
    Mutations causing proinsulin misfolding trigger unfolded protein response and lead to impaired proliferation and reduced mTORC1 signalling of developing beta-cells in a patient-derived induced pluripotent stem cell disease model.
    1. Cell Biology

    PDIA1/P4HB is required for efficient proinsulin maturation and ß cell health in response to diet induced obesity

    Insook Jang et al.
    The major protein disulfide isomerase family member, PDIA1, is essential in beta cells of mice fed a high-fat diet to maintain glucose homeostasis, proinsulin maturation and organelle integrity.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Mitochondria supply ATP to the ER through a mechanism antagonized by cytosolic Ca2+

    Jing Yong et al.
    ATP enters the endoplasmic reticulum (ER) lumen through an SLC35B1/AXER-dependentCaATiER mechanism, and ATP usage in the ER renders 'anti-Warburg' effect by increasing ATP regeneration from OxPhos while decreasing glycolysis.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Misfolded proteins bind and activate death receptor 5 to trigger apoptosis during unresolved endoplasmic reticulum stress

    Mable Lam et al.
    Death receptor 5 can directly sense misfolded proteins downstream of the endoplasmic reticulum to provide a quality control mechanism that executes apoptosis and prevents further production of misfolded proteins.
    1. Developmental Biology

    Inhibiting the integrated stress response pathway prevents aberrant chondrocyte differentiation thereby alleviating chondrodysplasia

    Cheng Wang et al.
    Integrated stress response-induced expression of ATF4 and its transactivation of SOX9 causes aberrant chondrocyte differentiation and skeletal defects which can be alleviated by modulating initiation-factor eIF2α phosphorylation translation control.
    1. Biochemistry and Chemical Biology
    2. Structural Biology and Molecular Biophysics

    An unfolded protein-induced conformational switch activates mammalian IRE1

    G Elif Karagöz et al.
    ER-stress sensing mechanism of the unfolded protein response sensor/transducer IRE1 is conserved from yeast to mammals, where in mammals, unfolded protein binding to IRE1's ER lumenal domain is coupled to its oligomerization and activation through an allosteric conformational change.
    1. Cell Biology

    The unfolded protein response in fission yeast modulates stability of select mRNAs to maintain protein homeostasis

    Philipp Kimmig et al.
    A unique form of regulation has been observed in the unfolded protein response of S. pombe, along with a novel mechanism of post-transcriptional mRNA processing.

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