25 results found
    1. Biochemistry and Chemical Biology

    The glucuronyltransferase B4GAT1 is required for initiation of LARGE-mediated α-dystroglycan functional glycosylation

    Tobias Willer et al.
    Post-phosphoryl modification of α-dystroglycan requires the glucuronyltransferase B4GAT1; this enzyme synthesizes the acceptor glycan that serves as a primer for the glycosyltransferase LARGE to synthesize the laminin-binding glycan.
    1. Biochemistry and Chemical Biology

    B4GAT1 is the priming enzyme for the LARGE-dependent functional glycosylation of α-dystroglycan

    Jeremy L Praissman et al.
    The correct enzymatic activity of a previously misnamed enzyme is defined, placing the enzyme upstream of LARGE in building functional O-mannose structures on α-dystroglycan that are disrupted in multiple forms of congenital muscular dystrophy.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Golgi self-correction generates bioequivalent glycans to preserve cellular homeostasis

    Haik Mkhikian et al.
    Structurally dissimilar N-glycans can be functionally equivalent, and are produced via a self-correcting feature of the Golgi.
    1. Biochemistry and Chemical Biology

    The functional O-mannose glycan on α-dystroglycan contains a phospho-ribitol primed for matriglycan addition

    Jeremy L Praissman et al.
    Disruption of the LG domain-binding phospho-ribitol-containing O-mannose structures on α-dystroglycan results in congenital muscular dystrophy.
    1. Cancer Biology

    Use of signals of positive and negative selection to distinguish cancer genes and passenger genes

    László Bányai et al.
    In contrast with earlier conclusions, negative selection has a major role in cancer evolution.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    The autophagy adaptor NDP52 and the FIP200 coiled-coil allosterically activate ULK1 complex membrane recruitment

    Xiaoshan Shi et al.
    Hydrogen-deuterium exchange, electron microscopy, and vesicle reconstitution show how binding of the autophagy adaptor NDP52 to the FIP200 subunit of the ULK1 complex triggers membrane binding in autophagy.
    1. Computational and Systems Biology
    2. Evolutionary Biology

    Deep evolutionary analysis reveals the design principles of fold A glycosyltransferases

    Rahil Taujale et al.
    Deep mining of GT-A fold sequences provides an evolutionary framework for investigating complex relationships connecting GT-A fold sequence, structure, function and regulation.
    1. Developmental Biology
    2. Medicine

    CHARGE syndrome modeling using patient-iPSCs reveals defective migration of neural crest cells harboring CHD7 mutations

    Hironobu Okuno et al.
    Neural crest cells differentiated from patient-derived cells with mutations in the chromatin remodeler CHD7 show defective delamination, migration and motility in vitro, and defective migration in chick embryos.
    1. Cell Biology

    Nuclear receptor LRH-1/NR5A2 is required and targetable for liver endoplasmic reticulum stress resolution

    Jennifer L Mamrosh et al.
    LRH-1/NR5A2 responds to ER stress by inducing a novel pathway that is required for stress resolution in mice and can be targeted by LRH-1 agonists.
    1. Genetics and Genomics
    2. Neuroscience

    Histone deacetylase knockouts modify transcription, CAG instability and nuclear pathology in Huntington disease mice

    Marina Kovalenko et al.
    Genetic knockout of Hdac2 modifies molecular and cellular phenotypes in Huntington’s disease mice and has a prominent transcriptional regulatory role in adult medium spiny neurons.

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