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Biomedical subjects

T Miyatake

Publications and source records attributed to T Miyatake.

At least 217 records · Page 12Linked to original sources
PubMed1990

[Auditory brainstem response and somatosensory evoked potential in Machado-Joseph disease in Japanese families].

To clarify physiological aspects of Machado-Joseph disease (MJD), we studied auditory brainstem response (ABR) and somatosensory evoked potential (SEP) in 17 clinically diagnosed patients with MJD aged 32-64 in Japanese families. ABR was recorded in 13 patients. In 8 patients, ABR were abnormal. In 5 patients, the latency of I wave was normal, but other waves could not be evoked. In the other 3 patients, I-III interpeak latency was prolonged. SEP was recorded in 15 patients. In SEP of median nerve, 11 patients had abnormal findings, and SEP of posterior tibial nerve revealed abnormal findings in all 15 patients. In all patients, responses from Erb's point and popliteal fossa were normal in latency, but other peaks were low in amplitude or absent, and the latency and central conduction time (CCT) were prolonged. The result of ABR indicated the involvement of the brainstem auditory pathways in MJD, and the result of SEP suggested that somatosensory pathways, particularly central pathways, would be involved in the disease process. ABR and SEP can be potential diagnostic methods for detection of subclinical abnormality in MJD patients.

Adult↗
PubMed1990

[An acute axonal form of Guillain-Barré syndrome with antibodies against gangliosides GM1 and GD1b--a case report].

We reported a case of Guillain-Barré syndrome with autoantibodies against gangliosides GM1 and GD1b, which has not been reported yet. A 25-year-old man was admitted with a 7-day history of acute progressive weakness in the extremities. Two weeks before admission he had suffered from an episode of watery diarrhea. Neurological examination revealed areflexia and tetraparesis without any sensory impairment. Respiration and cranial nerves were not involved. The CSF protein level was 157 mg/dl with normal cellularity on the 14th illness day. The maximum M potential amplitude was 0.1 mV, which was 2% of the lower limit of normal, whereas motor and sensory nerve conduction velocities were normal on day 22. Repeated electrophysiological studies suggested that the predominant process was axonal degeneration. Although he received plasmapheresis at the acute phase, four months after onset he continued to have distal dominant limb weakness with wasting. High-performance thin-layer chromatography with immunostaining revealed that his serum IgG reacted with GM1, GD1b, and asialo-GM1. Enzyme-linked immunosorbent assay showed that anti-glycolipids antibodies titers decreased, concurrent with clinical course. Immunoabsorption study demonstrated that antibodies with anti-GM1 activity were absorbed with liposomes containing purified GD1b, indicating that autoantibodies bind to the galactosyl (beta 1-3) N-acetylgalactosaminyl moiety which is shared by GM1, GD1b, and asialo-GM1. There have been several reports of lower motor neuron diseases with monoclonal IgM antibody to GM1, GD1b, and asialo-GM1. This case suggested that the transient rise in these anti-carbohydrate antibodies may be involved in the pathogenesis of acute axonal degeneration of motor nerve as well as paraproteinemic motor neuron diseases.

Acute Disease↗
PubMed1989

cDNA cloning of mouse myelin-associated glycoprotein: a novel alternative splicing pattern.

The structures of three forms of mouse myelin-associated glycoprotein mRNAs were determined from full-length cDNA clones. Two forms of mRNAs have been reported to be different by alternate inclusion of exon 2 and 12 in rat brain. One of the three forms of clones obtained here appeared to be a novel mRNA which lacked both the exon 2 and 12 portions, although others were identical splicing patterns to those of rat. Northern blot analysis using specific probes to mRNAs with or without the exon 2 portion in normal and quaking mouse confirmed that the splicing of exon 2 and 12 occurred independently.

Amino Acid Sequence↗
PubMed1989

cDNA cloning and amino acid sequence for human myelin-associated glycoprotein.

cDNA clones of human myelin-associated glycoprotein were isolated and analyzed. The combination of the two overlapping cDNA clones covered the full coding region and the complete amino acid sequence was deduced. In rat and mouse, expression of the two forms of mRNA is developmentally regulated; the mRNA without exon 12 portion is expressed mainly in the actively myelinating stage of development. Although the cDNA library used here was prepared from adult human brain poly(A)+ RNA, all five clones obtained corresponded to the mRNA without exon 12 portion.

Amino Acid Sequence↗
PubMed1989

Molecular cloning of a full-length cDNA for human alpha-N-acetylgalactosaminidase (alpha-galactosidase B).

In the process of molecular cloning of cDNA for proteins associated with a purified human placental sialidase fraction, we discovered one of the proteins with apparent molecular weight of 46 kDa is in reality alpha-N-acetylgalactosaminidase. The full length cDNA, pcD-HS1204, codes for 358 amino acids with the first 17 residues representing a putative signal peptide. The predicted amino acid sequence shows striking homology with human alpha-galactosidase A and yeast alpha-galactosidase. The substrate specificities as well as the behavior of the 46 kDa protein on hydroxylapatite chromatography confirmed that the 46 kDa protein is in reality alpha-N-acetylgalactosaminidase.

Amino Acid Sequence↗
PubMed1989

Accumulation of unique globo-series glycolipids in PC 12h pheochromocytoma cells.

In a previous paper, we reported the presence of a unique globo-series glycolipid as one of the major neutral glycolipid: Gal alpha 1-3Gal alpha 1-4Gal beta 1-4Glc beta 1-1' Cer, in the subcloned PC 12h pheochromocytoma cells (Ariga, T., Yu, R. K., Scarsdale, J. N., Suzuki, M., Kuroda, Y., Kitagawa, H., and Miyatake, T. (1988) Biochemistry 27, 5335-5340). Recently we found that the subcloned PC 12h cells accumulated other unusual neutral glycolipids. In order to characterize these glycolipids, PC 12h cells were subcutaneously transplanted into rats. The induced tumor tissue accumulated four minor neutral glycolipids, which were purified by droplet counter-current, Iatrobeads column, and preparative thin-layer chromatographies. These glycolipid structures were determined by fast atom bombardment-mass spectrometry, proton nuclear magnetic resonance spectroscopy, permethylation study, and sequential degradation with various exoglycosidases to be as follows: A, Fuc alpha 1-2Gal alpha 1-3Gal alpha 1- 4Gal beta 1-4Glc beta 1-1'Cer; B, GalNAc beta 1-3Gal alpha 1-3Gal alpha 1- 4Gal beta 1-4Glc beta 1-1'Cer; C, Gal alpha 1-3Gal alpha 1-3Gal alpha 1- 4Gal beta 1-4Glc beta 1-1'Cer; and D, Gal alpha 1-3Gal alpha 1-3Gal alpha 1- 3Gal alpha 1-4Gal beta 1-4Glc beta 1-1'Cer. Glycolipids A and B were tentatively characterized in normal rat small intestine (Breimer, M. E., Hansson, G. C., Karlsson, K.-A., and Leffler, H. (1982) J. Biol. Chem. 257, 557-568; Angstrom, J., Breimer, M. E., Falk, K.-E., Hansson, G. C., Karlsson, K.-A., and Leffler, H. (1982) J. Biol. Chem. 257, 682-688). Glycolipids C and D have not been reported in the literature.

Adrenal Gland Neoplasms↗
PubMed1989

NMR study on [1-13C]glucose metabolism in the rat brain during hypoxia.

The metabolism of [1-13C]glucose in the rat brain during hypoxia was investigated by 13C NMR spectroscopy. Male Wistar rats, weighing 100-120g, were anesthetized with ketamine (50 approximately 75 mg/kg i.p.) and ventilated mechanically with a mixture of 30% oxygen, 69.5% nitrogen and 0.5% halothane. [1-13C]glucose (250 mg/kg) was infused twice, at 10 minute intervals, through the femoral vein. For the control group (n = 4), the oxygen concentration of the inspiratory gas was maintained at 30% by vol throughout the experiments. For the hypoxia group (n = 6), the oxygen concentration in the inspiratory gas was reduced to 6-7% (93-94% nitrogen) and maintained for 30 min following [1-13C]glucose infusion. 13C NMR spectra were measured by a gated proton-decoupling method without a nuclear Overhauser effect. The [1-13C]glucose infusion gave apparent signals of the C1 carbon in the alpha- and beta-anomers of [1-13C]glucose at 92.7 and 96.7 ppm, respectively. Signals of the C2, C3 and C4 carbon atoms in glutamate and/or glutamine (glx) also appeared at 55, 27 and 34 ppm, respectively. The intensity of glx-C2 and glx-C3 signals increased later than that of glx-C4. The time lag between the different glx signals may reflect the turnover rate of the TCA cycle. Under the hypoxic condition, the signal of C3 carbon in lactate appeared at 21 ppm and increased. The alpha-glucose signal diminished during hypoxia, whereas the beta-glucose signal kept its intensity. The difference in changes of the signal intensity between alpha- and beta-glucose suggests that alpha-glucose is consumed more than beta-glucose in the hypoxic brain.

Animals↗
PubMed1989

Polymorphic sparteine metabolism and amyotrophic lateral sclerosis.

The activity of detoxication with the cytochrome P450 family of enzymes was studied in patients with amyotrophic lateral sclerosis (ALS). The metabolic ratio (MR) of sparteine sulphate was measured, and the population of phenotypes for sparteine nitro (N)-oxidation was determined in 30 patients with ALS and 41 controls for the assay of the sparteine N-oxidation by the cytochrome P450 enzymes. A lower MR and higher frequency of efficient metabolizers were observed in the ALS group, suggesting that there is efficient sparteine N-oxidation by cytochrome P450 pathways in ALS. This tendency was more marked in 15 patients with ALS aged under 60 years.

Adult↗
PubMed1989

Accumulation of glycosphingolipids in spinal and sympathetic ganglia of a symptomatic heterozygote of Fabry's disease.

Fabry's disease is an X-linked disorder of glycolipid catabolism. We have found a symptomatic heterozygous female with cardiomyopathy and severe pain in the extremities. We studied histochemically and biochemically the accumulated glycolipids in spinal and sympathetic ganglia of the patient. Histochemical examination demonstrated the marked glycolipid deposits that have been observed in heterozygous males in these ganglia. Gas-liquid chromatography (GLC) revealed that these accumulated glycolipids were characterized as globotriaosylceramide (Gb3cer) and galabiosylceramide (Ga2cer). In the heterozygous female, the accumulations of Gb3cer in spinal and sympathetic ganglia were, respectively, 34 and 48 times the amount in normal controls. This is the first report on quantitative and qualitative analyses of the accumulated glycolipids in spinal and sympathetic ganglia of a heterozygous carrier female.

Fabry Disease↗
PubMed1989

Developmental expression of 2',3'-cyclic-nucleotide 3'-phosphodiesterase mRNA in brains of normal and quaking mice.

A cDNA fragment of mouse 2',3'-cyclic-nucleotide 3'-phosphodiesterase was isolated and used as the probe for Northern blot analysis. The mRNA bands in normal mice became detectable at 10 days after birth, reached the maximum at the period of active myelination and then decreased gradually. At all stages of development studied, the mRNA bands in quaking mice were markedly reduced as compared with those in normal mice.

2',3'-Cyclic-Nucleotide Phosphodiesterases↗
PubMed1989

Antibodies to human spinal cord proteins in sera from patients with motor neuron disease and other neurological diseases.

Antibodies to human spinal-cord proteins in sera from patients with motor neuron disease (MND) and other neurological diseases were detected by the immunoblotting method. IgG in serum from a patient with amyotrophic lateral sclerosis reacted with insoluble proteins in the crude nuclear subfraction but the IgG in serum from other patients reacted with soluble proteins. The molecular weights of the antigens on the blots differed among the cases. The significance of the antibodies to neural tissue in sera from MND remains unknown. The etiology of MND is thought to be heterogenous and some types of MND may be closely related to humoral immunity.

Antibodies↗
PubMed1989

A Creutzfeldt-Jakob disease agent (Echigo-1 strain) recovered from brain tissue showing the 'panencephalopathic type' disease.

We used histologic evidence of degenerative changes in both the gray and white matter of the brain to diagnose a patient as having the panencephalopathic type of Creutzfeldt-Jakob disease (CJD). This type of CJD is relatively common in Japan, but not in North America or Europe. We recovered a transmissible pathogen (Echigo-1 strain) from an autopsy specimen of the patient's brain and passed it serially in Hartley guinea pigs. After a long latent period, it caused degenerative changes, mainly in the thalamic area of the guinea pig brain. On the 4th passage, a substrain emerged with a short latent period. When cross-transmitted to Golden Syrian hamsters, this substrain induced severe degeneration in both the thalamus and cerebral cortex. We compare our results with those for other experimental CJDs produced by other types of this disease.

Adult↗
PubMed1989

[Late-infantile form galactosialidosis with psychomotor retardation and spastic paraparesis].

We described a case of late-infantile form of galactosialidosis. This male patient was a product of normal pregnancy. His parents were first cousins. He first sat at eight months, walked and talked at two years of age. His gait gradually became unsteady and he was diagnosed as spastic paraparesis at the age of five years. Abnormally slow learning was first pointed out at seven years of age. At the age of nine years, we evaluated him in detail at our university hospital. Physical examination revealed a short stature for his age, slightly coarse face, short neck, funnel chest, genu, pes and hallucis valgus. Corneal clouding, hernia and angiokeratoma were not found. Neurological examination showed mental retardation, bilateral optic atrophy without cherry-red spots, and spastic and slightly ataxic gait. Slight muscular atrophy with weakness was also seen in the extremities, more remarkable in the lower limbs. Deep tendon reflexes were hyperactive with bilateral ankle clonus and no extensor planter response. Routine examination of blood, urine and cerebrospinal fluid were normal except for approximately 10% lymphocytes containing cytoplasmic vacuoles. X-ray films of the backbone exhibited vertebral plana with anterior breaking at the second lumbar vertebra level. The electroencephalography showed the multiple spike and slow wave complexes. Brain CT depicted the atrophy of cerebellum. The activities of sialidase and beta-galactosidase were markedly reduced in white blood cells and cultured skin fibroblasts in this patient. His urinary excretion of sialyloligosaccharides increased.(ABSTRACT TRUNCATED AT 250 WORDS)

Carbohydrate Metabolism, Inborn Errors↗
PubMed1989

[Juvenile Sandhoff disease with local panatrophy--a case report].

A case of juvenile Sandhoff disease with Gowers' local panatrophy was reported. A mentally retarded 39 year-old man had been noticed to have localized skin atrophy on the right leg since 7 years of age, and was also aware of the atrophy of his right leg since his junior high school age. His parents were first cousins. He had multiple localized atrophic skin lesions on the right side of his nape, the right side of lower back, and the right thigh. These skin lesions did not show any signs of sclerosis. Although he was mentally retarded (IQ=44), the neurological examination was normal including funduscopy. Histological examination of the atrophic skin le led that the epidermal basal layer was hyperpigmented and that the dermis and subcutaneous fatty tissue were extremely atrophic. The nerves meandered across the dermis and subcutaneous tissue, and had membranous lipid storage cytosomes in the axon and Schwann cells. Biochemical studies revealed marked deficiency fo leukocyte hexosaminidase activity. Total hexosaminidase activity was decreased to 18% of normal controls, and hexosaminidase B activity was completely deficient. Other lysosomal enzymes in leukocytes were normal in activities. Both parent showed intermediate levels of leukocyte hexosaminidase A and B activities. Electron microscopy analysis of a rectal biopsy specimen showed neuronal accumulation of dense osmophilic deposits, as well as membranous cytoplasmic bodies in Meissner's plexus. On the basis of the patient's age at onset and the above findings, the patient was diagnosed to have juvenile Sandhoff disease. The local panatrophy observed in the present case could be the result of the accumulation of GM2-ganglioside in autonomic nervous system, and may well be a new clinical feature of GM2-gangliosidosis.

Adult↗
PubMed1989

[Acute autonomic and sensory neuropathy associated with galactorrhea-amenorrhea syndrome and intractable anorexia].

Acute autonomic and sensory neuropathy (AASN), one subtype of acute pandysautonomia, in which dorsal root ganglia and autonomic ganglia are involved is uncommon. Little is so far known on central nervous system involvement in AASN. In the present paper we described a rare case of AASN associated with the central nervous system manifestations such as galactorrhea-amenorrhea syndrome and intractable anorexia. A 30-year-old woman rapidly developed burning pain and numbness in her arms and legs as well as orthostatic syncope. She had severe anorexia and no no menstruation from onset. On physical examination, she was emaciated. There was marked orthostatic hypotension with tachycardia. Skin was dry. Moderate galactorrhea was detected. Neurological examination showed prominent paresthesia and dullness of superficial sensation, predominantly to pinprick and thermal stimuli, segmentally over the neck, occipital scalp, and extremities. Deep sensation was intact. She had no weakness or ataxia. Deep tendon reflexes were almost normal. NCV and SEP were normal, while EEG was abnormal. Sural nerve biopsy demonstrated axonal degeneration with the loss of myelinated, predominantly in small-caliber fibers, and unmyelinated fibers. The levels of HVA and MHPG in CSF were decreased. The autonomic nervous function tests revealed postganglionic dysfunction. alpha-adrenergic system was predominantly impaired, while beta-adrenergic system was relatively preserved. The endocrinological studies demonstrated mild or moderate elevation of PRL basal value and hyper-response of PRL and LH for TRH and LH-RH loading test, which suggested disorder of the hypothalamo-hypophysial system. Cranial MRI showed moderate dilatation of the 3rd ventricle.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗
PubMed1989

[Subacute sensory neuropathy manifestated by tonic pupils associated with small cell carcinoma of the lung].

Only a few cases of carcinomatous neuropathy with tonic pupils have been reported. In the present paper we described a 53-year-old woman with subacute sensory neuropathy who had presented with bilateral tonic pupils. She noticed numbness over the medial aspect of the left thigh in March, 1988, and then developed dysesthesia over the left forearm and hand, mild weakness of left upper and lower extremities and urinary disturbance. Neurological symptoms were subacutely progressive and she was bed-ridden in May. She was admitted to our hospital in June, 1988. On examination, she had body-weight loss of 6 kg during the last six months and general status was otherwise unremarkable. She had anisocoria; the left pupil was larger in daylight than the right, while smaller in dim light. The left pupil scarcely reacted to light, but promptly constricted to near vision. The right pupil constricted normally to light and near vision. An instillation of 0.0625% pilocarpine solution showed supersensitive response of both pupils. An instillation of 1.25% epinephrine solution demonstrated mild dilation of both pupils. Thus, it was conceivable that she had postganglionic ciliary nerve damage characteristic of tonic pupil as well as the lesion of sympathetic nerve innervating pupillary dilator. She had severe sensory ataxia and pseudoathetosis of the hands. Weakness was mild to moderate in extremities. Almost all deep tendon reflexes were absent. All modalities of sensation, particularly on deep sense, were severely involved with sea-level-type distribution below Th7, and over C2 to C3 regions on the left side.(ABSTRACT TRUNCATED AT 250 WORDS)

Autonomic Nervous System Diseases↗
PubMed1989

[A case of amyotrophic lateral sclerosis associated with clinical features of progressive supranuclear palsy].

A 64-year-old male patient of amyotrophic lateral sclerosis (ALS) with frozen gait, axial rigidity and supranuclear upper gaze palsy was reported. We have followed this patient more than four years. He was well until November 1982, when he noticed weakness of left arm. In March 1983, he noticed hypogeusia and in July, he developed dysarthria and frozen gait. On admission, he was alert and oriented. Neurological examination revealed dysarthria, dysphagia and muscular weakness and atrophy in bilateral upper extremities, dominantly in left side. He showed remarkable frozen gait, retropulsion and could not walk. Brain CT showed mild dilatation of the third ventricle. In August 1988, he showed tongue atrophy, and weakness and atrophy of the extremities progressed during these four years. He also showed axial rigidity and frozen gait. Brain CT showed severe third ventricular dilatation and atrophy of tegmentum of the midbrain and cerebellum that were compatible with progressive supranuclear palsy (PSP). Six months later, he developed upper gaze palsy. From these findings, we concluded that this patient had a quite unique clinical features of both ALS and PSP.

Amyotrophic Lateral Sclerosis↗