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Article
LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy
This study finds a direct correlation between LARGE-glycan extension on dystroglycan and the protein’s capacity for extracellular matrix ligands; in regenerating mouse muscle, short LARGE-glycan polysaccharide...
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Article
Open AccessFukuyama-type congenital muscular dystrophy and defective glycosylation of α-dystroglycan
Fukuyama-type congenital muscular dystrophy (FCMD) is a severe form of muscular dystrophy accompanied by abnormalities in the eye and brain. The incidence of FCMD is particularly high in the Japanese populatio...
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Article
Association of dystroglycan and laminin-2 coexpression with myelinogenesis in peripheral nerves
To provide clues to the biological functions of dystroglycan–laminin-2 complex in peripheral nerves, we investigated the expressions of Β-dystroglycan and laminin-Α2 chain in rat sciatic nerve during axonal degen...
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Article
An ancient retrotransposal insertion causes Fukuyama-type congenital muscular dystrophy
Fukuyama-type congenital muscular dystrophy (FCMD), one of the most common autosomal recessive disorders in Japan (incidence is 0.7–1.2 per 10,000 births), is characterized by congenital muscular dystrophy ass...
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Article
Topographic pattern of the rearrangement of the dystrophin gene in Japanese Duchenne muscular dystrophy
To compare the frequency and distribution of rearrangements in the dystrophin gene in Duchenne muscular dystrophy (DMD) between Japanese DMD patients and those in North America and Europe, Southern blot analys...
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Article
Overexpression of dystrophin in transgenic mdx mice eliminates dystrophic symptoms without toxicity
DUCHENNE and Becker muscular dystrophy (DMD and BMD) are X-linked recessive diseases caused by defective expression of dystrophin1,2. The mdx mouse, an animal model for DMD, has a mutation that eliminates express...
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Article
Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle
DYSTROPHIN is associated with a complex of muscle membrane (sarcolemmal) glycoproteins that provide a linkage to the extracellular matrix protein, laminin1–8. The absence of dystrophin leads to a dramatic reducti...
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Article
Deficiency of the 50K dystrophin-associated glycoprotein in severe childhood autosomal recessive muscular dystrophy
X-LINKED recessive Duchenne muscular dystrophy (DMD) is caused by the absence of dystrophin, a membrane cytoskeletal protein1,2. Dystrophin is associated with a large oligomeric com-plex of sarcolemmal glycoprote...
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Chapter and Conference Paper
Fabrication of High Tc Superconducting Films by Spray Pyrolysis
A 100K superconducting Bi(Pb)-Sr-Ca-Cu-O film was formed on a MgO (100) single crystal by a spray pyrolysis method. Fifteen hours heating of the as-sprayed film at 845°C in air was enough to give the supercond...