8 results found
    1. Biochemistry and Chemical Biology
    2. Human Biology and Medicine

    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. Biochemistry and Chemical Biology
    2. Human Biology and Medicine

    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. Human Biology and Medicine

    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. Neuroscience

    A dystonia-like movement disorder with brain and spinal neuronal defects is caused by mutation of the mouse laminin β1 subunit, Lamb1

    Yi Bessie Liu et al.
    A mouse with a defined mutation in an extracellular matrix protein that is expressed in selected neurons sheds light on circuit abnormalities producing transient hyperkinetic movements.
    1. Cell Biology
    2. Developmental Biology

    Thrombospondin-4 controls matrix assembly during development and repair of myotendinous junctions

    Arul Subramanian, Thomas F Schilling
    Functional attachments between muscles and tendons require pentameric Thrombospondin-4, revealing novel roles both as an integrin ligand and extracellular matrix scaffold.
    1. Neuroscience

    Slit2 as a β-catenin/Ctnnb1-dependent retrograde signal for presynaptic differentiation

    Haitao Wu et al.
    A novel retrograde signal is involved in nerve terminal differentiation at mouse neuromuscular junctions.
    1. Neuroscience

    Injury-induced perivascular niche supports alternative differentiation of adult rodent CNS progenitor cells

    Justyna Ulanska-Poutanen et al.
    Distinctive microenvironments within demyelinated white matter determine fate decisions of adult oligodendrocyte progenitor cells.
    1. Biochemistry and Chemical Biology
    2. Structural Biology and Molecular Biophysics

    Structural basis of diverse membrane target recognitions by ankyrins

    Chao Wang et al.
    The 24 ankyrin repeats of ankyrin proteins form an extended solenoid that provides an extremely conserved groove for binding to numerous targets via combinatorial usage of multiple weak interaction sites.

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