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Y Sunada

Publications and source records attributed to Y Sunada.

At least 19 recordsLinked to original sources
PubMed2006

Inhibitors of the TGF-beta superfamily and their clinical applications.

The transforming growth factor-beta (TGF-beta) superfamily includes TGF-betas, activin, myostatin and bone morphogenetic proteins. Misregulation of the activity of TGF-beta family members is involved in pathogenesis of cancer, muscular dystrophy, obesity and bone and tooth remodeling. Natural inhibitors for the TGF-beta superfamily regulate fine-tuning of activity of TGF-beta family in vivo. In addition to natural inhibitors for the TGF-beta family, soluble forms of receptors for the TGF-beta family, blocking monoclonal antibodies and small chemical TGF-beta inhibitors have been developed. In this review, we summarize recent advances in our understanding of inhibitors for the TGF-beta superfamily and their medical applications.

Animals↗
PubMed2005

Reduced amplitude of the sural nerve sensory action potential in PARK2 patients.

The authors performed nerve conduction studies in nine PARK2 and eight idiopathic Parkinson disease patients and found a significant reduction of sural sensory nerve action potential (SNAP) amplitude in eight PARK2 patients who mostly remained asymptomatic. These data suggest that sensory axonal neuropathy may be a common clinical feature of PARK2 and a reduced amplitude of sural SNAP could be a diagnostic indicator of PARK2.

Action Potentials↗
PubMed2001

Alkynethiolato and alkyneselenolato ruthenium half-sandwich complexes: synthesis, structures, and reactions with (eta(5)-C(5)H(5))(2)Zr.

Alkynethiolato and alkyneselenolato complexes of ruthenium, CpRu(PPh(3))(2)(EC(triple bond)CR) (Cp = eta(5)-C(5)H(5); E = S, R = Ph (1a), SiMe(3) (1b), (t)Bu (1c); E = Se, R = Ph (2a), SiMe(3) (2b)), were synthesized by the reactions of CpRuCl(PPh(3))(2) with corresponding lithium alkynechalcogenolates in THF. An analogous reaction of Cp*RuCl(PEt(3))(2) (Cp* = eta(5)-C(5)Me(5)) with LiSC(triple bond)CPh produced Cp*Ru(PEt(3))(2)(SC(triple bond)CPh) (3). Complexes 1a and 2a were allowed to react in THF with "Cp(2)Zr", generated in situ from Cp(2)ZrCl(2) and 2 equiv of n-BuLi, from which the S-bridged Ru-Zr dinuclear complexes CpRu(PPh(3))(C(triple bond)CPh)(mu-S)ZrCp(2) (4a) and CpRu(PPh(3))(C(triple bond)CPh)(mu-Se)ZrCp(2) (4b) were isolated, respectively. In these complexes, C-S(Se) bond cleavage of the alkynechalcogenolate ligands was promoted by "Cp(2)Zr", and the Zr atom was oxidized from II to IV. Treatment of 4a and 4b in THF under 1 atm CO gave rise to CpRu(CO)(C(triple bond)CPh)(mu-E)ZrCp(2) (E = S (5a), Se (5b)), while addition of tert-butyl isocyanide to a THF solution of 4b afforded CpRu(CN(t)()Bu)(C(triple bond)CPh)(mu-Se)ZrCp(2) (6). The crystal structures of 1a, 1c, 2a, 2b, 3, 4a, 4b, and 5b were determined by X-ray diffraction analysis.

Journal Article↗
PubMed2001

Structural organization, complete genomic sequences and mutational analyses of the Fukuyama-type congenital muscular dystrophy gene, fukutin.

Fukuyama-type congenital muscular dystrophy (FCMD) is an autosomal recessive severe muscular dystrophy in combination with cerebral cortical dysplasia. Previously, we identified the gene responsible for FCMD, termed fukutin, through positional cloning. In this study, we have sequenced 131892 bp of genomic DNA in the region of the fukutin gene on chromosome 9q31 and obtained its complete genomic structure. The fukutin genomic sequence spans approximately 100 kb and is organized into 10 exons (41-6067 bp) and nine introns (1841-21460 bp). Using these sequence data, we have identified three novel fukutin mutations in FCMD patients. We have also located a putative TATA box in the flanking 5' region and identified numerous alternatively spliced fukutin mRNA transcripts. Analysis of expressed sequence tag clusters within the region revealed two novel genes upstream of the fukutin gene. These data provide fundamental information to support detailed genetic and functional analyses of the fukutin gene.

Alternative Splicing↗
PubMed2001

Transgenic mice expressing mutant caveolin-3 show severe myopathy associated with increased nNOS activity.

Caveolin-3 is the muscle-specific isoform of the caveolin protein family, which is a major component of caveolae, small membrane invaginations found in most cell types. Caveolins play important roles in the formation of caveola membranes, acting as scaffolding proteins to organize and concentrate lipid-modified signaling molecules, and modulate a signaling pathway. For instance, caveolin-3 interacts with neuronal nitric oxide synthase (nNOS) and inhibits its catalytic activity. Recently, specific mutations in the caveolin-3 gene, including the Pro104Leu missense mutation, have been shown to cause an autosomal dominant limb-girdle muscular dystrophy (LGMD1C), which is characterized by the deficiency of caveolin-3 in the sarcolemma. However, the molecular mechanism by which these mutations cause the deficiency of caveolin-3 and muscle cell degeneration remains elusive. Here we generated transgenic mice expressing the Pro104Leu mutant caveolin-3. They showed severe myopathy accompanied by the deficiency of caveolin-3 in the sarcolemma, indicating a dominant negative effect of mutant caveolin-3. Interestingly, we also found a great increase of nNOS activity in their skeletal muscle, which, we propose, may play a role in muscle fiber degeneration in caveolin-3 deficiency.

Animals↗
PubMed2001

[LGMD1B(lamin A/C)].

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Chromosomes, Human, Pair 1↗
PubMed2001

[LGMD1D].

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Cardiomyopathy, Hypertrophic↗
PubMed2001

[Sequential magnetic resonance imaging and neurophysiological studies in a patient with inferior spinal cord infarction].

A 66-year-old woman suddenly developed anterior spinal artery syndrome with complete flaccid paraplegia, superficial sensory disturbance caudally to the L5 dermatome level with preservation of deep sensation, incontinence, and absent deep tendon reflexes in both legs. An MRI of the whole spine and an analysis of the CSF 4 hours after onset were normal. The electrophysiological study showed an absence of F wave on the posterior tibial nerve stimulation on admission, while the peripheral nerve conduction velocities and amplitudes of upper and lower limbs were normal. T2-weighted MRI 4 days after onset demonstrated an area of high signal intensity in the gray and white matters of the epiconus and conus medullaris, and T1-weighted MRI showed the swelling. Three weeks later, F wave became evoked nd the high signal areas on axial T2-weighted MRI were localized in the bilateral anterior horns of the gray matter. T1-weighted MRIs after an administration of Gd-DTPA 3 and 7 weeks after onset demonstrated an enhancement of the ventral roots of the lumbar nerves and cauda equina, while the enhancements disappeared 8 months after onset. The patient was finally able to walk independently over 10 meter. An absent F wave was an only positive finding at the hyperacute (hours after onset) stage of the spinal cord infarction.

Aged↗
PubMed2000

Expression of dystroglycan and laminin-2 in peripheral nerve under axonal degeneration and regeneration.

In Schwann cells, the transmembrane glycoprotein beta-dystroglycan composes the dystroglycan complex, together with the extracellular glycoprotein alpha-dystroglycan which binds laminin-2, a major component of the Schwann cell basal lamina. To provide clues to the biological functions of the interaction of the dystroglycan complex with laminin-2 in peripheral nerve, the expression of beta-dystroglycan and laminin-alpha2 chain was studied in rat sciatic nerves undergoing axonal degeneration and regeneration as well as in normal condition. In normal sciatic nerve, immunoreactivity for the cytoplasmic domain of beta-dystroglycan was consistently and selectively localized in the Schwann cell cytoplasm underlying the outer (abaxonal) membrane apposing the basal lamina. While beta-dystroglycan expression was gradually down-regulated in Schwann cells losing contact with axons during axonal degeneration, it was progressively up-regulated as the regenerating process of ensheathment and myelination proceeded during regeneration. Interestingly, beta-dystroglycan expression, when detectable, was always restricted to the Schwann cell cytoplasm beneath the outer membrane apposing the basal lamina during both axonal degeneration and regeneration. Furthermore, laminin-alpha2 immunoreactivity roughly paralleled that of beta-dystroglycan during both axonal degeneration and regeneration, indicating that the expression of beta-dystroglycan and laminin-alpha2 is induced and maintained by the Schwann cell contact with axons. Our results indicate that the dystroglycan complex is involved in the adhesion of the Schwann cell outer membrane with the basal lamina and suggest that the dystroglycan complex may play a role in the process of Schwann cell ensheathment and myelination through the interaction with laminin-2.

Animals↗
PubMed2000

Identification of a methylated tea catechin as an inhibitor of degranulation in human basophilic KU812 cells.

We examined the constituents of tea that had an inhibitory effect on the degranulation process in the human basophilic cell line, KU812. Among the constituents purified from a extract of 'Benihomare' oolong tea by column chromatography, a methylated (-)-epigallocatechin gallate ((-)-epigallocatechin 3-O-(3-O-methyl) gallate) was found to inhibit the degranulation of KU812 cells that had been stimulated with calcium ionophore A23187. The inhibitory effect of this methylated (-)-epigallocatechin gallate on degranulation was greater than that of (-)-epigallocatechin gallate. This result indicates that methylation of (-)-epigallocatechin gallate may be an effective modification for the catechin to inhibit degranulation from human basophils.

Basophils↗