M Shimmoto
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Explore the profile of M Shimmoto including associated specialties, affiliations and a list of published articles.
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34
Citations
255
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Recent Articles
1.
Naganawa Y, Itoh K, Shimmoto M, Takiguchi K, Doi H, Nishizawa Y, et al.
J Hum Genet
. 2000 Aug;
45(4):241-9.
PMID: 10944856
To gain insight into the pathogenesis of sialidosis type 1, we performed molecular investigations of two unrelated Japanese patients. Both of them are compound heterozygotes for base substitutions of 649G-to-A...
2.
Takiguchi K, Itoh K, Shimmoto M, Ozand P, Doi H, Sakuraba H
J Hum Genet
. 2000 Aug;
45(4):200-6.
PMID: 10944848
To clarify the molecular basis of the late infantile form of galactosialidosis, we characterized a defective protective protein/cathepsin A (PPCA) gene product with the K453E mutation newly found in an...
3.
Itoh K, Oyanagi K, Takahashi H, Sato T, Hashizume Y, Shimmoto M, et al.
Ann Neurol
. 2000 Jan;
47(1):122-6.
PMID: 10632112
Endothelin-1 is a peptidic substrate in vitro of lysosomal protective protein/cathepsin A (PPCA) with serine carboxypeptidase activity. Endothelin-1-specific immunoreactivity has been demonstrated to be markedly increased and distributed abnormally in...
4.
Naganawa Y, Itoh K, Shimmoto M, Kamei S, Takiguchi K, Doi H, et al.
Biochem J
. 1999 May;
340 ( Pt 2):467-74.
PMID: 10333491
Fibroblastic cell lines derived from a galactosialidosis patient, stably expressing the chimaeric green fluorescent protein variant (EGFP) gene fused to the wild-type and mutant human lysosomal protective protein/cathepsin A (PPCA)...
5.
Utsumi K, Itoh K, Kase R, Shimmoto M, Yamamoto N, Katagiri Y, et al.
Clin Chim Acta
. 1999 Mar;
279(1-2):55-68.
PMID: 10064118
A renal disorder is one of the important manifestations of Fabry disease, but the details of the pathogenesis have not been clarified yet. We examined the possibility that the vitronectin...
6.
Sakuraba H, Itoh K, Shimmoto M, Utsumi K, Kase R, Hashimoto Y, et al.
Neurology
. 1999 Feb;
52(2):372-7.
PMID: 9932959
Objective: To determine the clinical features and biochemical basis of the first Japanese patient with the GM2 gangliosidosis AB variant. Methods: The clinical manifestations and laboratory findings in the patient...
7.
Itoh K, Naganawa Y, Kamei S, Shimmoto M, Sakuraba H
Biochem Biophys Res Commun
. 1999 Jan;
253(2):228-34.
PMID: 9878520
The 32/20-kDa two-chain form of protective protein/cathepsin A (CathA) secreted by human platelets was thermostable in the aggregation supernatant at acidic pH, but was denatured at neutral pH. Leupeptin partly...
8.
Itoh K, Shimmoto M, Utsumi K, Mizoguchi N, Miharu N, Ohama K, et al.
Biochem Biophys Res Commun
. 1998 Jun;
247(1):12-7.
PMID: 9636645
Galactosialidosis is a human autosomal recessive lysosomal storage disease caused by a genetic defect of protective protein/cathepsin A (PPCA). The patients in a Japanese family with the severe early-infantile form...
9.
Kase R, Shimmoto M, Itoh K, Utsumi K, Kotani M, Taya C, et al.
Biochim Biophys Acta
. 1998 Jun;
1406(3):260-6.
PMID: 9630664
Human lysosomal alpha-galactosidase predominantly hydrolyzes ceramide trihexoside. A transgenic mouse line, C57BL/6CrSIc-TgN(GLA) 1951 Rin, highly expressing human alpha-galactosidase, has been established and investigated biochemically and immunohistochemically in order to clarify...
10.
Hara A, Uyama E, Uchino M, Shimmoto M, Utsumi K, Itoh K, et al.
J Neurol Sci
. 1998 Apr;
155(1):86-91.
PMID: 9562328
We describe a 31-year-old Japanese man with adult Sandhoff s disease presenting as spinocerebellar degeneration. There was a marked cerebellar atrophy on MRI, and proliferation of abundant PAS-positive foamy macrophages...