Area of research
Biochemistry · Molecular Biology
Research interest
Research interests include Sulfur Compounds in Biology, Glutathione Transferases and Polymorphisms, Muscle Physiology and Disorders, and Amino Acid Enzymes and Metabolism.
LARGE1 processively polymerizes length-controlled matriglycan on prodystroglycan
N-terminal domain on dystroglycan enables LARGE1 to extend matriglycan on α-dystroglycan and prevents muscular dystrophy
Identification of a short, single site matriglycan that maintains neuromuscular function in the mouse
<i>Large1</i> gene transfer in older <i>myd</i> mice with severe muscular dystrophy restores muscle function and greatly improves survival
Structure and mechanism of LARGE1 matriglycan polymerase
Dystroglycan N-terminal domain enables LARGE1 to extend matriglycan on α-dystroglycan and prevents muscular dystrophy
Muscular dystrophy-dystroglycanopathy in a family of Labrador retrievers with a LARGE1 mutation
POMK regulates dystroglycan function via LARGE1-mediated elongation of matriglycan
HNK-1 sulfotransferase modulates α-dystroglycan glycosylation by 3-O-sulfation of glucuronic acid on matriglycan
Clinical utility of RNA sequencing to resolve unusual GNE myopathy with a novel promoter deletion
Protective role for the N-terminal domain of α-dystroglycan in Influenza A virus proliferation
Exogenous expression of the glycosyltransferase LARGE1 restores α-dystroglycan matriglycan and laminin binding in rhabdomyosarcoma
Biochemical and pathological changes result from mutated Caveolin-3 in muscle
Structural basis of laminin binding to the LARGE glycans on dystroglycan
Role of dystroglycan in limiting contraction-induced injury to the sarcomeric cytoskeleton of mature skeletal muscle
Structure of protein O-mannose kinase reveals a unique active site architecture
Endogenous Glucuronyltransferase Activity of LARGE or LARGE2 Required for Functional Modification of α-Dystroglycan in Cells and Tissues
SGK196 Is a Glycosylation-Specific <i>O</i> -Mannose Kinase Required for Dystroglycan Function
Dystroglycan Function Requires Xylosyl- and Glucuronyltransferase Activities of LARGE
Xylosyl- and glucuronyltransferase functions of LARGE in α-dystroglycan modification are conserved in LARGE2
RUI: Exploring Synthesis, Tailoring Structure, Evaluating Properties, and Enabling Patterning of Surface-Anchored Framework Assemblies
RUI: Enabling Rational Design of Smart Interfaces Incorporating Metal-Organic Coordinated Assemblies
RUI: Enabling Rational Design of Smart Interfaces Incorporating Metal-Organic Coordinated Assemblies
Japanese Language Fellowship for Erik K. Webb
Scaling Problems in Hydrogeological Modeling
Mathematical Models of Groundwater-Surface Water Systems