394 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. 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. 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. Biochemistry and Chemical Biology
    2. Cell Biology

    The Lamin B receptor is essential for cholesterol synthesis and perturbed by disease-causing mutations

    Pei-Ling Tsai et al.
    Lamin B receptor may provide a long-sought model system enabling unprecedented studies of protein quality control in the nuclear envelope of mammalian cells.
    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

    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. 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.
    1. Biochemistry and Chemical Biology

    Cell Biology: Opening up new fronts in the fight against cholesterol

    Russell A DeBose-Boyd, Jay D Horton
    Insight
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    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Full length RTN3 regulates turnover of tubular endoplasmic reticulum via selective autophagy

    Paolo Grumati et al.
    Full length RTN3 homodimerization mediates ER tubules fragmentation and their subsequent delivery to lysosome.

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