[Giant axonal neuropathy--comparing with acrylamide poisoning].
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Acrylamides↗
Biomedical subjects
I Goto
Publications and source records attributed to I Goto.
[Midazolam for rapid sequence induction].
We compared midazolam 0.2 mg.kg-1 and fentanyl 50 micrograms with thiamylal 4 mg.kg-1 for rapid sequence induction. We could use midazolam safely in patients with bronchial asthma or drug allergy. There was no difference in time from the beginning of induction to intubation between midazolam treated group and thiamylal treated group. Changes in systolic as well as diastolic blood pressure and heart rate during 2 hours from intubation were smaller in midazolam treated group than in thiamylal treated group. In midazolam treated group, no arrhythmias were observed at the time of intubation. We could reduce the amount of anesthetics in midazolam treated group during 2 hours from intubation. From the results mentioned above, we conclude that midazolam is a useful agent for rapid sequence induction.
Adult↗
[Reduction in leukocyte elastase levels in patients with sepsis by administration of urinastatin].
Leukocyte elastase (LE) plays a role in the development of shock. Therefore, we examined the effects of urinastatin, a protease inhibitor, on LE in 7 patients with sepsis. LE levels before the administration of urinastatin were high in all patients, especially those in shock. Administration of urinastatin markedly decreased LE levels, with the decrease being more remarkable in patients showing high LE levels before treatment. These results suggest that urinastatin not only reduces LE levels but also suppresses factors that are involved in the development of septic shock.
Adult↗
[A case of juvenile Sandhoff disease].
A Japanese male with juvenile Sandhoff disease is described. The patient was a product of full-term normal pregnancy from non-consanguineous parents. Since age 10, he developed progressive dysarthria and proximal muscle atrophy and weakness. Mental deterioration and cerebellar ataxia are also noted since the age of 20. On neurological examination at age 35, he showed decreased mentality (IQ 62), marked atrophy and weakness of proximal muscles, cerebellar ataxia and increased deep tendon reflexes. Brain CT scans revealed moderate to marked atrophy of cerebellum. Giant MUP, fasciculation potentials and positive sharp waves were observed on EMG examination. Biopsied sural nerve showed markedly decreased myelinated fibers. Hexosaminidase A and B activities in leukocytes and cultured fibroblasts were about 10% of normal values, while other lysosomal enzyme activities were within normal range. Rectal biopsy demonstrated lamellar inclusion bodies in submucosal ganglion cells. This is the first Japanese patient with juvenile Sandhoff disease presenting symptoms similar to motor neuron disease and cerebellar degeneration.
Adult↗
[A case of demyelinating disease showing a peculiar honeycomb-like and lamellar structure on magnetic resonance imaging].
A case of subacute demyelinating disease showing large honeycomb-like and lamellar lesions in bilateral cerebral white matter on MRI was reported. A 22 year-old woman felt general fatigue on sept. 30, 1988, and developed complete quadriplegia and mutism in about 10 days. She recovered shortly after high-dose corticosteroid therapy. She had neither fever nor meningeal irritation. There were no inflammatory changes in examinations of blood and CSF. On MRI, high and low signal areas of irregular shape in the cerebral white matter and brainstem, were observed on T2 and T1 weighted images, respectively, suggesting that the lesions are of demyelinating nature. These lesions appeared lamellar or honeycomb-like structures on T1 and T2 weighted image. This patient was diagnosed as having a subacute type of demyelinating disease similar to Balo's disease (concentric sclerosis).
Adult↗
Metabolism of free sphingoid bases in murine tissues and in cultured human fibroblasts.
Metabolism of free sphingoid bases present in normal tissues [Kobayashi, T., Mitsuo, K. & Goto, I. (1988) Eur. J. Biochem. 172, 747-752] was examined in mouse tissues and in human cultured fibroblasts. Subcellular fractionation studies of the mouse liver revealed most of free sphingoid bases to be in the membrane fractions. Fibroblasts from patients with Farber's disease contained concentrations of free sphingoid bases similar to those seen in the normal fibroblasts. When L-cycloserine, a potent inhibitor of the first reaction of sphingoid base synthesis, was added to the culture medium, the concentration of free sphingoid bases in fibroblasts decreased, dose-dependently. Thus, most of the free sphingoid bases in the tissue probably do not derive from the degradation of sphingolipids but are newly synthesized. Free sphingoid bases in microsomes from the brainstem and spinal cord were acylated or glycosylated when incubated with acyl-CoA or UDP-glycoside. However, the reaction for the synthesis of ceramide was much greater and more rapid than that of glycosylsphingosine synthesis. In liver microsomes, ceramide synthesis from endogenous free sphingoid bases was observed but synthesis of glycosylsphingosine was not evident. Therefore, the main pathway of metabolism of free sphingoid bases is presumably acylation (to ceramide) but not glycosylation (to lysoglycosphingolipid).
Animals↗
The twitcher mouse: attenuated processes of Schwann cells in unmyelinated fibers.
Morphological alterations occurring in Schwann cells of unmyelinated fibers (unmyelinated Schwann cells) were investigated in the sciatic nerve of the twicher mouse, a murine model of human globoid cell leukodystrophy. After postnatal day 10, the number of Schwann cell-axon units gradually increased and the number of unmyelinated axons per unit progressively decreased in the twitcher mouse. However, the total number of unmyelinated axons showed no significant differences between twitcher and normal mice. Thus, these alterations of unmyelinated Schwann cells in the twitcher mouse suggest that attenuated branching of cellular processes develops at an early stage and progresses together with progression of demyelination in this mutant.
Animals↗
Sensitive determination of ambenonium chloride in serum from patients with myasthenia gravis using ion-exchange resin extraction and reversed-phase ion-pair chromatography.
An effective and selective procedure for the extraction of ambenonium chloride (AMBC) from serum using a weak cation-exchange extraction cartridge has been developed. The solid-phase extraction procedure permitted the extraction of AMBC from serum without adhesion to materials such as the containers. A 200-microliter volume of the eluate could be directly injected on to a reversed-phase ion-pair high-performance liquid chromatographic column. The recovery was in the range 97-100%. The limit of detection for AMBC was 0.5 ng/ml in serum (signal-to-noise ratio = 3). The method was used to determine the serum concentration of AMBC in patients with myasthenia gravis. The method would be useful for monitoring AMBC in serum in order to study its pharmacokinetic behaviour in patients under oral administration therapy.
Adult↗
Generation of CD4+ blastoid T cells showing marked upregulation of CD4, class I and II MHC, and IL2 receptor molecules is required for the expression of potent encephalitogenicity.
The relationship between surface molecule expression and encephalitogenicity of myelin basic protein (BP)-sensitized cells induced by three different sensitization protocols was studied using adoptive transfer in Lewis rats. (i) In BP/CFA sensitization, CD4+ blastoid T cells showing marked upregulation of CD4, class I and II MHC, and IL2 receptor molecules, but not CD5, CD8, or CD45, were generated after culture with BP. In this case, BP-cultured cells were strongly encephalitogenic in the recipients. (ii) In the case of BP/IFA sensitization, CD4+ T cells showed no remarkable change of cell size or surface molecule expression after culture with BP and were weakly encephalitogenic in the recipients. Vigorous proliferation of the cells induced by addition of recombinant IL2 to the culture with BP neither enhanced the encephalitogenicity nor produced CD4+ blastoid T cells showing marked upregulation of CD4, class I and II MHC, and IL2 receptor molecules. (iii) The sequentially transferred naive T cells showed no remarkable change of cell size or surface molecule expression, even after a second culture with BP, and were the least encephalitogenic. These data suggest that the generation of CD4+ blastoid T cells showing marked upregulation of CD4, class I and II MHC, and IL2 receptor molecules but not vigorous proliferation correlates closely with the potent encephalitogenicity in vivo.
Animals↗
Tetramer formation of a variant type human transthyretin (prealbumin) produced by Escherichia coli expression system.
A variant of human transthyretin(TTR, prealbumin) with methionine for valine substitution at position 30 is a major component of amyloid fibrils found in patients of familial amyloidotic polyneuropathy(FAP) type I, an autosomal dominant genetic disease. But the molecular nature of the variant TTR has been obscure, because most of plasma TTR from FAP patients is a mixture of variant and wild type TTR and no pure preparation of the variant has been available. For this reason, we constructed a system in which the variant type TTR was efficiently synthesized. In this system, the recombinant variant TTR was first synthesized as a fusion protein with E. coli outer membrane protein A (ompA) signal peptide, processed to eliminate the signal peptide and finally secreted to the culture medium. The final concentration of the recombinant variant TTR in the medium was about 5 mg/l. SDS polyacrylamide gel electrophoresis and gel filtration analysis suggested that the recombinant variant TTR can form tetramer as seen for native one. Purification of the protein was accomplished by only two steps of chromatography.
Blotting, Western↗
The twitcher mouse: accumulation of galactosylsphingosine and pathology of the central nervous system.
In the twitcher mouse, a murine model of globoid cell leukodystrophy (GLD), pathological changes of various parts of the central nervous system correlated well with the concentration of galactosylsphingosine (psychosine). The development of GLD lesions was more obvious in tracts with a more rapid progression of myelination. It was suggested that accumulation of galactosylsphingosine subsequent to myelin maturation caused suicidal death of myelin forming cells.
Aging↗
Accumulation of lysosulfatide (sulfogalactosylsphingosine) in tissues of a boy with metachromatic leukodystrophy.
Abnormal accumulation of lysosulfatide (sulfogalactosylsphingosine) was evident in autopsied tissues from a boy with late-infantile metachromatic leukodystrophy. The concentration was high in the cerebral white matter, spinal cord and sciatic nerve (116-787 pmol/mg protein) and low in the cerebral gray matter, kidney and liver (4-40 pmol/mg protein). As is the case with galactosylsphingosine, lysosulfatide inhibited cytochrome c oxidase activity, in a dose-dependent manner. Judging from the tissue distribution of the accumulated lysosulfatide and because of the cytotoxicity, the lysosulfatide presumably explains the demyelination seen in the nervous tissues of patients with metachromatic leukodystrophy.
Animals↗
Structure and expression of the alternatively-spliced forms of mRNA for the mouse homolog of Alzheimer's disease amyloid beta protein precursor.
The human amyloid beta protein (BP) is a major constituent of the amyloid deposited in the brain of patients with Alzheimer's disease and is derived from a larger precursor protein (BPP). In human three alternatively-spliced forms of BPP mRNA were found and two of them were shown to encode a protease inhibitory activity. We have isolated the corresponding species of cDNA in mice and found that the inhibitor domain is highly conserved through mammalian evolution. The homology between human and mouse was 94.6%. Northern blot using specific probes showed that the mRNA for BPP with inhibitor domain was present in every tissue, particularly at a higher level in the kidney. On the other hand, that without inhibitor domain was found most abundantly in the brain but much less in the kidney and the intestine. These data suggest that the individual BPP mRNA species were produced in a tissue-specific manner in mouse as in the case of human.
Alzheimer Disease↗
Increases in helper inducer T cells and activated T cells in HTLV-I-associated myelopathy.
Using two-color flow cytometric analysis, we studied peripheral blood lymphocyte subsets in 15 patients with human T-cell lymphotropic virus type I-associated myelopathy. The percentage of CD4+ 4B4+ cells (helper inducer T cell) was significantly increased in the patients with the myelopathy, compared with 16 healthy control subjects who were seronegative for human T-cell lymphotropic virus type I. However, there was no difference in the percentage of CD4+ 2H4+ cells (suppressor inducer T cell) between the two groups. The ratio of CD4+ 4B4+ cells to CD4+ cells and CD4+ 4B4+ to CD4+ 2H4+ cells was also elevated in these patients. The percentage of CD4+ DR+ cells and CD8+ DR+ cells, both of which are phenotypically activated T cells, and the ratio of CD4+ DR+ cells to CD4+ cells and of CD8+ DR+ cells to CD8+ cells are also increased in the patients, compared with the control subjects. The percentage of CD4+ 4B4+ cells showed positive correlations with values of spontaneous proliferation of peripheral blood lymphocytes and serum IgG level in patients with the myelopathy.
Adult↗
Activated T cells in HTLV-I-associated myelopathy: autologous mixed lymphocyte reaction.
We examined the autologous mixed lymphocyte reaction in human T-cell lymphotropic virus type I-associated myelopathy to elucidate the known increase in spontaneous proliferation of peripheral blood lymphocytes. Proliferative responses in the autologous mixed lymphocyte reaction test were significantly increased (34,205 cpm) in cells from 9 patients with the myelopathy, as compared with findings (18,695 cpm) in the control subjects. When non-T-cell fractions were depleted from autologous mixed lymphocyte reaction cultures, proliferative responses were completely suppressed in the control subjects, while proliferation of the peripheral blood lymphocytes from the groups with the myelopathy were not affected by depletion of non-T-cell fractions. The increase in spontaneous proliferation of peripheral blood lymphocytes in patients with the myelopathy is probably not related to an increase in autologous mixed lymphocyte reactions.
Adult↗
Familial myopathy with scapulohumeral distribution, rigid spine, cardiopathy and mitochondrial abnormality.
A 37-year-old woman with scapulohumeral muscular atrophy, rigid spine and cardiopathy is reported. Muscle weakness, advanced atrioventricular block and contractures at the neck, elbows and ankles had occurred during her childhood. An autosomal dominant mode of inheritance was suggested because her mother, sister and brother had the same disorder. Pleomorphic mitochondria had accumulated in the subsarcolemmal space of the skeletal muscle. There was no evident enzyme defect in the mitochondrial electron transport system. Although the clinical features had some similarity with those of Emery-Dreifuss muscular dystrophy or rigid spine syndrome, the pattern of inheritance and the muscle pathology differed.
Adult↗
Congenital myopathy with myasthenic features and congenital cataract in two siblings.
Two siblings with congenital myopathy showing myasthenic manifestations together with congenital cataract are reported. Their muscle weakness fluctuated and was alleviated by edrophonium chloride. Their serum creatine kinase activity was elevated, and the waning phenomenon was observed on repetitive nerve stimulation. Biopsied muscle showed degenerative changes with type 1 fibre predominance and abnormal morphology of neuromuscular junctions.
Adult↗
Acrylamide and thiamine.
Acrylamide administration to rats caused clear alteration of blood thiamine content, which appeared to reflect suppressed intake and/or utilization of the vitamin in the body. Decreased utilization of thiamine does not seem to be induced by decreased binding of thiamine pyrophosphate (TPP) and apotransketolase, because acrylamide did not affect the Km of TPP for transketolase activity.
Acrylamides↗