859 results found
    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

    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. Neuroscience

    Defective synaptic transmission causes disease signs in a mouse model of juvenile neuronal ceroid lipofuscinosis

    Benedikt Grünewald et al.
    Impaired GABAergic and glutamatergic synaptic function and loss of interneurons in the amygdala, hippocampus, and cerebellum cause characteristic disease symptoms in a mouse model juvenile neuronal ceroid lipofuscinosis.
    1. Cell Biology

    A new family of StART domain proteins at membrane contact sites has a role in ER-PM sterol transport

    Alberto T Gatta et al.
    A new family of sterol-specific lipid transfer proteins has been found that anchors in the endoplasmic reticulum; some of these proteins stretch across membrane contacts and mediate sterol traffic from the plasma membrane.
    1. Neuroscience

    Steroid hormone induction of temporal gene expression in Drosophila brain neuroblasts generates neuronal and glial diversity

    Mubarak Hussain Syed et al.
    A steroid hormone acts directly on brain neural progenitors to coordinately alter gene expression and specify neuronal and glial cell types.
    1. Neuroscience

    Developmental NMDA receptor dysregulation in the infantile neuronal ceroid lipofuscinosis mouse model

    Kevin P Koster et al.
    NMDA receptor function is regulated by protein depalmitoylation during visual cortical maturation and is dysfunctional in infantile neuronal ceroid lipofuscinosis.
    1. Biochemistry and Chemical Biology

    Exchange of water for sterol underlies sterol egress from a StARkin domain

    George Khelashvili et al.
    The capture and export of a hydrophobic sterol molecule from the binding pocket of a Lam/GramD1 sterol-binding protein is unexpectedly mediated by water.
    1. Cell Biology
    2. Evolutionary Biology

    Sterol transfer by atypical cholesterol-binding NPC2 proteins in coral-algal symbiosis

    Elizabeth Ann Hambleton et al.
    Diversification of a conserved cholesterol binder drives functional replacement of cholesterol with symbiont-produced sterols in corals living in nutrient-poor environments.
    1. Developmental Biology
    2. Neuroscience

    Mitochondrial fusion but not fission regulates larval growth and synaptic development through steroid hormone production

    Hector Sandoval et al.
    The dual role of Drosophila Mitofusin in steroid hormone production and cholesterol ester storage, which is evolutionary conserved by the combined expression of the two mammalian Mitofusins, ensures proper synaptic development.
    1. Neuroscience

    A Drosophila model of neuronal ceroid lipofuscinosis CLN4 reveals a hypermorphic gain of function mechanism

    Elliot Imler et al.
    Genetic analysis of a CLN4 Drosophila model suggests that the disease-causing alleles act as dominant gain of function mutations that cause CSPα oligomerization and impair secretory and prelysosomal trafficking.

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