330 results found
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Doa10 is a membrane protein retrotranslocase in ER-associated protein degradation

    Claudia C Schmidt et al.
    Doa10, a membrane-embedded ubiquitin ligase, facilitates the removal of membrane proteins from the endoplasmic reticulum and cooperates with the Cdc48 ATPase in this process.
    1. Cell Biology

    USP13 antagonizes gp78 to maintain functionality of a chaperone in ER-associated degradation

    Yanfen Liu et al.
    A deubiquitinase antagonizes the activity of an associated E3 ligase to prevent uncontrolled ubiquitination of a degradation machinery protein and thus maintain its functionality in protein quality control at the endoplasmic reticulum.
    1. Developmental Biology
    2. Immunology and Inflammation

    Notch-induced endoplasmic reticulum-associated degradation governs mouse thymocyte β−selection

    Xia Liu et al.
    Endoplasmic reticulum-associated degradation (ERAD) is a critical protein quality control checkpoint for thymocyte β-slection.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Sterol homeostasis requires regulated degradation of squalene monooxygenase by the ubiquitin ligase Doa10/Teb4

    Ombretta Foresti et al.
    Parallel branches of the ERAD pathway regulate sterol synthesis at multiple steps via feedback inhibition.
    1. Developmental Biology

    Enhanced ER-associated degradation of HMG CoA reductase causes embryonic lethality associated with Ubiad1 deficiency

    Youngah Jo et al.
    Embryonic lethality associated with deficiency of UBIAD1, which synthesizes a vitamin K2 subtype, results from aberrant ER-associated degradation of the cholesterol biosynthetic enzyme HMG CoA reductase.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Schnyder corneal dystrophy-associated UBIAD1 inhibits ER-associated degradation of HMG CoA reductase in mice

    Youngah Jo et al.
    UBIAD1 mediates a unique geranylgeranyl pyrophosphate-sensing mechanism that when disrupted, inhibits degradation of HMG CoA reductase and triggers overproduction of corneal cholesterol that characterizes the eye disease Schnyder Corneal Dystrophy.
    1. Cell Biology
    2. Developmental Biology

    Regulation of BMP4/Dpp retrotranslocation and signaling by deglycosylation

    Antonio Galeone et al.
    The cytoplasmic enzyme N-glycanase 1 plays an evolutionary conserved role in promoting the ERAD-mediated retrotranslocation of misfolded Dpp/BMP4 from the ER, thereby allowing BMP signaling in specific contexts.
    1. Immunology and Inflammation

    RNF41 regulates the damage recognition receptor Clec9A and antigen cross-presentation in mouse dendritic cells

    Kirsteen M Tullett et al.
    Dendritic cell recognition and processing of antigens from dead cells, utilising the Clec9A-damage recognition receptor, is controlled by a novel RNF41-ubiquitin-mediated regulatory pathway.
    1. Cell Biology
    2. Genetics and Genomics

    A Drosophila screen identifies NKCC1 as a modifier of NGLY1 deficiency

    Dana M Talsness et al.
    An ion transporter is hyperglycosylated and 50% less functional in NGLY1-deficient cells, potentially explaining several symptoms of NGLY1 deficiency such as lack of sweat and tears.
    1. Cell Biology

    Splicing variation of BMP2K balances abundance of COPII assemblies and autophagic degradation in erythroid cells

    Jaroslaw Cendrowski et al.
    Erythroid-enriched BMP2K kinase, in addition to its predicted function in endocytosis, regulates distribution and abundance of COPII assemblies and autophagic degradation through opposing actions of its two splicing variants.

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