503 results found
    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. 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

    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

    The prenyltransferase UBIAD1 is the target of geranylgeraniol in degradation of HMG CoA reductase

    Marc M Schumacher et al.
    The inhibition of sterol-accelerated degradation of HMG CoA reductase by the vitamin K2 synthetic enzyme UBIAD1 may contribute to the accumulation of cholesterol that is associated with Schnyder corneal dystrophy.
    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

    The sterol-responsive RNF145 E3 ubiquitin ligase mediates the degradation of HMG-CoA reductase together with gp78 and Hrd1

    Sam A Menzies et al.
    CRISPR/Cas9 genome-wide screens using sterol-sensitive endogenous HMG-CoA reductase (HMGCR) reporter identify the sterol-responsive RNF145 and gp78 as independently responsible for sterol-accelerated degradation of HMGCR, the rate-limiting enzyme of cholesterol biosynthesis.
    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. Cancer Biology
    2. Genetics and Genomics

    FoxO suppresses endoplasmic reticulum stress to inhibit growth of Tsc1-deficient tissues under nutrient restriction

    Avantika Gupta, Hugo Stocker
    Transcriptomic and genetic analyses of FoxO target genes reveal a key function of ER-associated protein degradation in growth regulation of Tsc1-deficient tissues.

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