Kazutaka Shiomi

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We examined the characteristic clinical features of one family of familial amyotrophic lateral sclerosis (FALS) with a His46Arg mutation in the enzyme Cu/Zn superoxide dismutase-1 (SOD1). The disease duration for this family was 18.1 +/- 13.2 (mean +/- S.D.) years, with the age at onset being 39.7 +/- 10.5 years old (mean +/- S.D.). The initial sign was(More)
We studied 107 cases and 64 carriers of type I familial amyloidotic polyneuropathy (FAP) residing in 16 districts in Japan. The age of onset of illness ranged from 20 to 71 years old, with a mean of 40.1 +/- 12.8 years (SD). One quarter of the cases were late-onset patients who developed the disorder after age 50. Asymptomatic carriers older than age 50(More)
Human neutrophil peptides (HNPs) are 29 to 30 amino acid antimicrobial peptides localized in the azulophil granules in neutrophils. We isolated endogenous molecular forms of HNPs from normal human bone marrow, plasma and peripheral blood neutrophils and determined their primary structures to investigate their post-translational processing. HNPs initially(More)
Guanylin is a 15-amino acid peptide that acts on intestinal guanylate cyclase, thereby regulating intestinal fluid and electrolyte transport through the second messenger, cyclic GMP. Using synthetic rat guanylin, we prepared an antiserum that recognizes human and rat guanylin equally on a molar basis and developed a sensitive radioimmunoassay (RIA). The(More)
Neutrophil peptide, also called defensin, is an antimicrobial peptide localized in azurophil granules in neutrophils. Using a synthetic human neutrophil peptide-1 (HNP-1), we prepared an antiserum against the peptide and established a highly sensitive radioimmunoassay (RIA). This RIA equally recognized HNP-1, -2, and -3 on a molar basis. Analyses of(More)
We report a 49-year-old man who was a human T-cell leukemia virus type 1 (HTLV-1) carrier, born in Okinawa prefecture where both strongyloidiasis and HTLV-1 are endemic. He presented with fever, headache and urinary retention. On the basis of CSF examination and MRI findings, his condition was diagnosed as myelitis. He received methylprednisolone pulse(More)
We identified and determined the content and molecular form of islet amyloid polypeptide (IAPP/amylin) in the gastrointestinal (GI) tract of human, rat, mouse and cat. IAPP was isolated by anti- IAPP- IgG immunoaffinity chromatography and reverse-phase high performance liquid chromatography coupled with radioimmunoassays for human and rat/mouse IAPPs. Human(More)
Acute autonomic and sensory neuropathy is a rare disorder that has been only anecdotally reported. We characterized the clinical, electrophysiological, pathological and prognostic features of 21 patients with acute autonomic and sensory neuropathy. An antecedent event, mostly an upper respiratory tract or gastrointestinal tract infection, was reported in(More)
Using a synthetic N-terminal hexadecapeptide of islet amyloid polypeptide (IAPP), we prepared an antiserum specific for IAPP[1-16] and established an extremely sensitive radioimmunoassay (RIA) for the peptide with a minimum detection level of 0.26 fmol/tube. Since the N-terminal sequence of IAPP is 100% conserved in many mammalian species, the RIA is widely(More)