23 results found
    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 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
    2. Developmental Biology

    Cholesterol accessibility at the ciliary membrane controls hedgehog signaling

    Maia Kinnebrew et al.
    The patched hedgehog receptor inhibits the transmembrane transducer smoothened by reducing the accessibility of cholesterol locally at the membrane of the primary cilium.
    1. Computational and Systems Biology
    2. Genetics and Genomics

    Genetic effects on promoter usage are highly context-specific and contribute to complex traits

    Kaur Alasoo et al.
    Re-analysis of large gene expression datasets with state-of-the-art quantification methods reveals additional candidate genes mediating complex trait associations.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    SEC24A deficiency lowers plasma cholesterol through reduced PCSK9 secretion

    Xiao-Wei Chen et al.
    COPII vesicles regulate the metabolism of cholesterol through the selective transport of secretory proteins.
    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. Biochemistry and Chemical Biology
    2. Cell Biology

    Hedgehog signaling is a potent regulator of liver lipid metabolism and reveals a GLI-code associated with steatosis

    Madlen Matz-Soja et al.
    The Hedgehog signalling pathway is a master regulator of lipid metabolic processes and their zonation in the adult liver of mice and humans.
    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. Cell Biology
    2. Immunology and Inflammation

    Interaction mapping of endoplasmic reticulum ubiquitin ligases identifies modulators of innate immune signalling

    Emma J Fenech et al.
    Interaction mapping of ubiquitin ligase complexes embedded in the endoplasmic reticulum membrane has identified interactors of RNF26 that influence innate immune signalling.

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