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Biomedical subjects

T Kitaguchi

Publications and source records attributed to T Kitaguchi.

At least 19 recordsLinked to original sources

Neurite promoting activity of collagens on embryonic neurons: decreased effect at the postnatal stage.

We studied the in vitro neurite outgrowth activity of fibronectin, laminin, heparan sulfate proteoglycan, type I collagen, type IV collagen, and type VIII collagen in cholinergic neuronal cell lines and primary cultured neurons. All these substances had high neurite promoting activity on primary cultured neurons from embryonic mouse brain. However, collagens had no such an effect on primary cultured neurons from postnatal brain. When neuronal cell lines were used, collagens and other extracellular matrix substances were equally and highly effective on cells originated from embryonic brain, but collagens were less effective on cells from postnatal brain. These findings suggest that postnatal neurons lose the neuritic responsiveness to collagens earlier than that of other ECM.

Animals

Beta-protein immunoreactivity in brains of patients with neuronal ceroid lipofuscinosis: ultrastructural and biochemical demonstration.

The storage pigment in neuronal ceroid lipofuscinoses (NCL) has a close similarity to age pigment lipofuscin. We studied immunoreactivity of isolated neuronal pigments from the juvenile form of NCL and aging control, using monoclonal antibodies (mAbs) against amyloid beta-protein. Ultrastructural localization of the immunoreactivity demonstrated that in NCL the epitopes are distributed mainly in curvilinear multilamellar arrays of the storage pigments and less in fingerprint profiles, while in aging control they are more homogeneously distributed on age pigment lipofuscin. The different distribution of the epitopes may reflect some catabolic as well as morphologic differences in lysosomes. A unique 31-kDa polypeptide detected on Western blots in NCL possibly derives from the same precursor, amyloid beta-protein precursor (ABPP). ABPP processing may be aberrant in NCL brains, and this can be detected as a 31-kDa polypeptide reactive with the mAbs.

Adolescent

Topographic heterogeneity of amyloid B-protein epitopes in brains with various forms of neuronal ceroid lipofuscinoses suggesting defective processing of amyloid precursor protein.

To verify our hypothesis of defective protease inhibitor domains that are encoded by abnormal processing of amyloid precursor protein (APP) in brains of patients with neuronal ceroid lipofuscinoses (NCL), immunohistochemical and cytochemical studies were performed with monoclonal antibodies (mAbs) directed against various domains of APP. For the studies, 22 autopsy brains were used: 12 with different forms of NCL, and 10 control brains. The staining procedure for the avidin-biotin complex (ABC) technique and the postembedding gold-labelled procedure for electron microscopy (EM) were employed. Of all mAbs used for the study, only mAbs generated against amyloid B-protein bound to neural tissue were affected with NCL. The strongest immunostaining of neurons and of some reactive glial cells was found in brains with the juvenile form of NCL. Only in the infantile form of the disease were some neurons overloaded with storage material weakly immunoreactive. In brains of patients with the adult form of NCL, immunoreactivity was found in affected neurons and in extracellularly deposited material of senile plaques. The results of EM study showed that the immunoreactivity was restricted to lysosomal cytosomes in neural tissue with any form of NCL selectively localized on the curvilinear and fingerprint proteinaceous component of ceroid lipofuscin. Studies performed on control aging brains and Alzheimer's disease (AD) brains confirmed previous observations of immunoreactivity being found diffusely in the protein component of some neurons containing lipopigment.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Prevalences of malocclusions and dental caries in molars in female adolescents.

Prevalences of malocclusions and dental caries in molars were investigated on a database of high school students collected between 1977 and 1986. A total of 6,665 schoolgirls were evaluated. The proportions of 54.1% in the seventh grade and 56.6% in the tenth grade students were judged as having malocclusions. The prevalences of dental caries for the lower first and the second molars were significantly higher (p less than 0.001) than those for the upper opponents during the observation period. The prevalence of dental caries increased linearly for all kinds of molars in accordance with age, but they were lower than those reported by the Ministry of Health and Welfare in Japan determined for the entire nation. The percent of dental caries for the second molars revealed abrupt increases from 10% at the seventh grade to 50% at the 12th grade. These changes were particularly noticeable between the eighth and the tenth grades. These results suggest the significance of oral health care education during the adolescent period based on a long-term cooperation made by dentists, school nurses, teachers through the participation of students into the oral health care programme.

Adolescent

[Prevalence of malocclusions and demand for orthodontic treatment among students at a women's high school].

Prevalences of malocclusions were investigated in a sample of 3,520 girls recorded between 1984 and 1986 at a private high school. Also, proportions of students who had received and were under treatment were surveyed for 3,501 students in 1989. In addition, ratios of a number of those who were judged to reveal maxillary protrusion with respect to that of the whole sample examined were evaluated as a function of overjet. The results of the surveys are as follows. SURVEY I) A total prevalence of malocclusion was 57.9% in the Grade 7 (G7) and 56.4% in the Grade 10 (G10). Among malocclusions surveyed, maxillary protrusion was found in 8.1% of the G7 and in 7.8% of the G10. Deep overbite was determined in 7.9% of the G7 and in 7.6% of the G10, mandibular protrusion was found in 2.4% of the G7 and in 2.7% of the G10. Edge-to-edge incisor relationships were determined in 6.4% of the G7 and in 7.6% of the G10. Anterior open bite was found in 2.4% of the G7 and in 2.9% of the G10. Crowding was found in 37.3% of the G7 and in 33.9% of the G10. SURVEY II) Prevalence of students who had orthodontic treatment at the time of survey, was 6.4% in the G7, 7.0% in the G8, 6.0% in the G9, 6.0% in the G10, 5.4% in the G11, and 3.6% in the G12. Those who had had orthodontic treatment revealed proportions of 8.1% in the G7, 8.0% in the G8, 7.7% in the G9, 11.3% in the G10, 12.2% in the G11, and 13.7% in the G12 students. SURVEY III) A proportion of 73.5% of those who had overjets more than 6 mm were judged to have maxillary protrusion.

Adolescent

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

[A female case of carnitine palmitoyltransferase deficiency].

A 17-year old woman noted myalgia after taking a long distance walk at the age of 10. In adolescence, she had several episodes of myalgia and pigmenturia after athletic activity or infection. At age 17, myoglobinuria and abnormally increased serum creatine kinase were documented after one of these episodes. The neurological examination revealed mild proximal muscle weakness of upper extremities. Electromyography showed myogenic patterns, such as brief, small abundant potentials on them. Venous lactate was raised normally on the ischemic exercise test. During prolonged fasting, plasma ketone bodies increased normally but there were abnormal elevations of plasma creatine kinase and myoglobin. Morphometric analysis of electron microscopy in muscle showed few lipid deposits and that of light microscopy revealed no abnormality. CPT activity in muscle was only 15% of normal value by the isotope-exchange assay. These results were consistent with the diagnosis of CPT deficiency. Although several cases of CPT deficiency with recurrent myoglobinuria have been reported in Western countries, our patients is the first case of Japanese showing recurrent myoglobinuria. CPT deficiency should be considered as a differential diagnosis in cases of recurrent myoglobinuria.

Acyltransferases

Formalin fixed brains are useful for magnetic resonance imaging (MRI) study.

We carried out magnetic resonance imaging (MRI) studies on human brains which had been fixed in formalin solution for over 2 years and had been proven neuropathologically to be cases of multiple sclerosis (MS), progressive multifocal leukoencephalopathy (PML), and Balo's concentric sclerosis (Balo). Using spin echo (SE) and inversion recovery (IR) pulse sequences to detect demyelinated lesions in a living person with MS, the demyelinated lesions of the fixed brains in cases of MS, PML and Balo definitely re-appeared, although T1 and T2 in the gray and white matter were reduced following fixation. High signal areas on the SE images corresponded not only to the characteristic distribution of demyelinated lesions in the white matter but also to sparse myelin, gliosis and mild perivascular cuffing in the white matter around the demyelinated foci in cases of the fixed MS, PML and Balo brains. On the IR images, only MS plaques were evident. This MRI study of fixed brains proved useful to elucidate clinicopathological correlations.

Adolescent

Late infantile Krabbe leukodystrophy: MRI and evoked potentials in a Japanese girl.

A Japanese girl showed deterioration in development from the age of 13 months. At the age of 16 months, there were mild spastic diplegia, increase in cerebrospinal fluid protein to 61.5 mg/dl and deficient galactosylceramidase I. Magnetic resonance imaging (MRI) demonstrated a high signal intensity with increased T2 in the centrum semiovale. Short latency somatosensory evoked potentials (SSEPs) showed a prolonged N13-N20 interpeak latency followed by abolition of N20. Brainstem auditory evoked potentials (BAEPs) were normally followed by prolonged interpeak latencies of wave I-V. This may be the first report of what we consider to be the late infantile form of Krabbe disease with MRI and evoked potential examinations.

Brain

Nonfamilial prealbumin-type amyloid polyneuropathy.

A 53-year-old man with nonfamilial prealbumin-type amyloid polyneuropathy had severe motor, sensory, and autonomic polyneuropathy, beginning at age 48 years. These clinical features closely resembled familial amyloid polyneuropathy (FAP), but abnormal serum prealbumin levels, specific to FAP (Japanese type), were not detected by radioimmunoassay; DNA sequence for prealbumin was normal. Thus, the diagnosis of FAP was excluded. A possible diagnosis of systemic senile amyloidosis was also considered.

Amyloidosis

Familial spinocerebellar degeneration as an expression of adrenoleukodystrophy.

A family with adrenoleukodystrophy and clinical manifestations of spinocerebellar degeneration was studied. Two adult male first cousins showed progressive limb and truncal ataxia, slurred speech and spasticity of the extremities. Brain CT scans demonstrated atrophy of the pons and cerebellum, in both cases. Very long chain fatty acids in plasma and erythrocyte membranes were elevated in the affected patients and intermediately increased in an aunt and the mother of one patient, thereby indicating homozygotes and carriers of adrenoleukodystrophy, respectively. This unusual type of adrenoleukodystrophy seems to be transmitted as an X-linked recessive trait.

Adrenoleukodystrophy