Wiskott-Aldrich syndrome in two sisters.
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Female↗
Biomedical subjects
T Kondoh
Publications and source records attributed to T Kondoh.
Localization of dopamine receptors and associated mRNA in transplants of human fetal striatal tissue in rodents with experimental Huntington's disease.
Huntington's Disease (HD) is characterized by deficits in motor and cognitive functions. This neurodegenerative disease shows an extensive loss of medium-sized spiny projection neurons (GABAergic) within the neostriatum. With the loss of these neurons, there is a concomitant loss of associated receptors, such as those for GABA, glutamate, and dopamine. In the present study, we have addressed the question of whether dopamine receptors are re-established in the lesioned rodent striatum following the transplantation of human striatal cells. Human striatal cell suspension or saline (transplant controls) was injected into the striatum of rats previously lesioned with quinolinic acid (QA). Three nine months following transplantation, the animals were sacrificed and the brains were processed for receptor autoradiography and in situ hybridization of dopamine D1 and D2 receptor subtypes. Our results demonstrate that animals transplanted with human striatal cells show a significant increase in D1 receptors following transplantation when compared to the lesion area in control animals, while D1 receptor mRNA remains unchanged. In contrast to D1 receptor binding, D2 receptor levels are not increased in the lesioned and transplanted area of the striatum when compared to controls; however, D2 receptor mRNA levels are significantly increased. These results demonstrate that at the times the animals were examined, D1 and D2 receptors were differentially regulated. Our results further indicate that human striatal primordium will survive following transplantation and will express D1 receptors and D2 receptor mRNA that are depleted in the QA lesioned rodent striatum. This study compliments and extends previous findings on human striatal cell transplantation in rodent models of HD.
Animals↗
Assessment of left ventricular volume using ECG-gated SPECT with technetium-99m-MIBI and technetium-99m-tetrofosmin.
UNLABELLED: We evaluated ECG-gated SPECT (g-SPECT) in the measurement of absolute left ventricular (LV) volume by comparing it with left ventriculography (LVG) and with cine-MRI. METHODS: Projection data from 31 patients were acquired with a three-headed SPECT system in 12 min using a 64 x 64 matrix with 1.5 zoom (1 pixel = 4.27 mm). The R-R interval from simultaneously acquired ECG was divided into eight frames. The end-diastolic and end-systolic volumes (EDV; ESV) and LV mass were assessed by an area-length method with manual delineation of the epi- and endocardial LV borders using midventricular vertical and horizontal long-axis images. The stroke volume, LVEF and cardiac output (CO) were generated from the EDV, ESV and heart rate during the study. The g-SPECT LV values were compared with those of LVG (25 patients) and cine-MRI (18 patients). RESULTS: The g-SPECT values correlated well with those from LVG (r = 0.83 to 0.92; p < 0.001) and cine-MRI (r = 0.76 to 0.99; p < 0.001). The g-SPECT technique provides an assessment of LV volumes (EDV, ESV, stroke volume, LVEF, CO, LV mass). CONCLUSION: Despite potential problems that may cause inaccuracy and require improvements such as an accurate and reproducible automatic edge detection algorithm, g-SPECT has clinical utility in assessing global LV volumes and function.
Adult↗
[A case of traumatic extracranial internal carotid artery dissecting aneurysm treated by proximal ligation and STA-MCA bypass].
We present a case with the traumatic extracranial internal carotid artery dissecting aneurysm. A 21-year-old man was involved in a motorcycle accident, resulting in multiple injuries but no apparent head and neck injuries. Head CT was normal on his admission. He was discharged from his local hospital 3 weeks after the accident without any neurological deficits. Five weeks after the accident, he suddenly presented with a motor aphasia and a right hemiparesis. CT and MRI showed infarctions in the left para-Sylvian and the left angular areas. Angiography showed a left extracranial carotid artery dissecting aneurysm at the level of C1 vertebral arch. The patient was initially managed by an anticoagulant agent, but he suffered from another transient ischemic attack due to distal embolism from the aneurysm. Balloon occlusion test of the left ICA was performed under monitoring EEG, SEP. Mean stump pressure (MSP) revealed 60 mmHg. and MSP/Mean systematic blood pressure revealed 67%. We judged that the left ICA ligation was a safe method to treat this patient, however, considering the patient's age and the side of the lesion, left STA-MICA bypass and ligation of the left ICA were carried out in one stage. Postoperatively, the patient did not show any cerebral ischemic complications and angiography showed disappearance of the aneurysm and patency of the bypass. The left MCA territories were filled well by cross circulation and the bypass.
Accidents, Traffic↗
Contribution of chorda tympani and glossopharyngeal nerves to taste preferences of rat for amino acids and NaCl.
To learn how the gustatory nerves convey information about the nutritionally dependent taste preference, intake of amino acid solutions and saline in rats with bilateral chorda tympani (CTX) and/or glossopharyngeal neurotomy (GPX) was determined during the feeding of a control diet (C) and a L-lysine (Lys) deficient diet (LD). Intact rats preferred L-arginine (Arg) more in C and Lys more in LD. The CTX group did not select nor ingest Lys in LD, and its intake of Arg was also low in C. The GPX group did not substantially alter its preference under both diets, while it did show an increase in total liquid intake. The preference changes in the CTX + GPX group appeared as combined effects of the CTX and the GPX groups. In an additional study, the preference for Lys shifted to higher concentrations and the total consumption of Lys increased in LD. The present data suggest that the chorda tympani nerves possibly function as discriminators of the nutritional information by altering the taste preference, and that the glossopharyngeal nerves may convey other functional taste information, such as aversive tastes, and sensory aspects of osmotic regulation. In addition, it is revealed that the animals have ability to search for a nutrient deficient in their body, and to ingest it to a level that at least nullifies the deficiency.
Amino Acids↗
Transplantation of human fetal striatum into a rodent model of Huntington's disease ameliorates locomotor deficits.
Previous studies have demonstrated that syngeneic transplants of striatal tissue can ameliorate locomotor deficits in rodent models of Huntington's disease (HD). In the present study, we have examined whether human to rat xenografts of fetal striatal tissue can exert a similar recovery of function. Rodents with unilateral striatal lesions were transplanted with human striatal cells from a donor 14 weeks post-conception, and subsequently displayed a progressive decrease in rotational asymmetry in comparison to sham (saline) transplanted animals. Histological analysis revealed acetylcholinesterase (AChE)-positive fibers and NADPH-diaphorase (NADPH-d)-positive neurons within transplanted tissue. These results suggest that human fetal striatum at a gestational age of 14 weeks may potentially be useful as a source of donor tissue for transplantation in the treatment of HD.
Acetylcholinesterase↗
Transplantation of human striatal tissue into a rodent model of Huntington's disease: phenotypic expression of transplanted neurons and host-to-graft innervation.
The present study was undertaken to investigate the phenotypic expression and integration of human striatal neurons transplanted into an animal model of Huntington's disease. Sprague-Dawley rats were anesthetized and subjected to quinolinic acid lesions of the left striatum. Three human fetal cadavers were utilized for transplantation in this study (7, 8, and 10 weeks in gestation). The striatal primordia was dissected from each fetus and subsequently dissociated into cell suspensions. Following the initial lesion surgeries (3-4 months), the rats were reanesthetized and transplanted with human striatal cells (400,000 cells per rat). The animals were processed for histochemical analysis 9-17 weeks posttransplantation. Histochemistry was performed utilizing thionin (Nissl staining), acetylcholinesterase, NADPH-diaphorase, and antibodies against tyrosine hydroxylase and glial fibrillary acidic protein. Examination of stained brain sections demonstrate that human striatal transplants grow to fill a substantial portion of the remaining striatum, and contain clusters of immature and mature cells. Acetylcholinesterase activity is present in the transplant neuropil, varying in intensity, and distributed in a heterogeneous fashion. In addition, host afferent dopaminergic fibers penetrate into the transplant, and are occasionally found in patches. NADPH-diaphorase histochemistry revealed medium sized aspiny striatal neurons of donor origin in the transplants. The results of this study are similar to those obtained with rodent fetal striatal transplants, and suggest that human striatal tissue is capable of surviving, expressing normal striatal cell phenotypes, and receiving host dopaminergic innervation.
Acetylcholinesterase↗
Elevated levels of nitric oxide in synovial fluid from patients with temporomandibular disorders.
OBJECTIVE: The objective of this study was to measure nitric oxide activity in synovial fluid samples taken from patients with temporomandibular disorders. STUDY DESIGN: We investigated six volunteers without symptoms and 56 patients with temporomandibular disorders. Nitric oxide activity was measured in temporomandibular joint synovial fluid with a highly sensitive and specific chemiluminescence detection method. RESULTS: A detectable nitrite concentration was found in only 1 of 10 joints in the control group. Measurable levels (> 0.001 mol/l) of nitrites in the synovial fluid were found in 8 (72.7%) of 11 joints with disk derangement with clicking, 24 (85.7%) of 28 joints with disk derangement with locking, and all 26 (100%) joints with osteoarthritis. The mean nitrite concentrations in the temporomandibular joint synovial fluid in the disk derangement with clicking, disk derangement with locking, and osteoarthritis groups were significantly higher than that in the control group (p < 0.05, p < 0.001, p < 0.001, respectively). CONCLUSIONS: These data clearly show increased nitric oxide production in certain temporomandibular disorders and suggest that nitric oxide may play a role in the pathogenesis of synovitis and degenerative changes of cartilaginous tissue and bone of the temporomandibular joint.
Adolescent↗
Increased histidine preference during specific alteration of rhythm of environmental temperature stress in rats.
It has been reported that specific alteration of rhythm of environmental temperature (SART) stress induces various physiological changes. In this study, changes in taste preference during SART stress were investigated in rats. Rats were given free access to six amino acid solutions, saline, and water in a choice paradigm. During SART stress, daily food intake increased significantly by 50% whereas the rate of body weight gain decreased significantly to one third that observed during the prestress baseline period. In addition, consumption of histidine solution increased significantly, whereas intakes of water, monosodium glutamate, saline, glycine, arginine, lysine, and threonine were unaffected. Results suggest that a specific preference for histidine emerges during SART stress, which may be related to the stress-induced changes in the histamine turnover in the brain and peripheral tissues.
Acclimatization↗
Therapeutic time window for the 21-aminosteroid, U-74389G, in global cerebral ischemia.
A novel 21-aminosteroid (U-74389G), a new potent antioxidant, was evaluated for its protective effect on transient global cerebral ischemia. Ischemia was induced by 20 minutes of four-vessel occlusion in adult male Wistar rats. Injection of 21-aminosteroid (U-74389G, 5 mg/kg intraperitoneally injected) was repeated three times. The second injection was performed 30 minutes after the first injection, and the third injection was performed 210 minutes after that. Experimental animals were divided into five groups according to the time drug administration was initiated. Group I (n = 8) began vehicle administration 30 minutes before occlusion. Group II (n = 9) started 21-aminosteroid administration 30 minutes before occlusion. Drug administration in Group III (n = 9) began at the time of reperfusion, in Group IV (n = 8), 30 minutes after reperfusion, and in Group V (n = 6), 60 minutes after reperfusion. Animals in the control group (n = 5) underwent sham operations. One week after ischemia, the number of viable pyramidal neurons was counted in the hippocampal CA1 subfield. The results were as follows: the number of living neurons in Group I was 18.8 +/- 8.7; in Group II, was 44.7 +/- 9.5; in Group III, was 46.4 +/- 9.4; in Group IV, was 40.3 +/- 6.6; in Group V, was 10.2 +/- 2.5; and in the control group was 131 +/- 3.3. Groups II, III, and IV demonstrated significantly higher numbers of living neurons compared with Group I (P < 0.05). The present study revealed that U-74389G attenuated delayed neuronal death in global cerebral ischemia when it was administered before or soon after the ischemic episode.
Animals↗
[Transsphenoidal surgery for a case of empty sella syndrome associated with GH secreting pituitary adenoma].
A 33-year-old woman was admitted to our hospital with acromegalic face as her chief complaint. Her neurological examination was normal, and endocrinological examination revealed a high level of growth hormone (GH) (12.8 ng/ml). CT cisternography and MRI showed an enlarged empty sella and a pituitary tumor. We performed a transsphenoidal approach operation to remove the tumor and to repair the empty sella. The tumor, which was compressed to the lateral and posterior wall of the sella turcica by the empty sella, was totally removed by meticulous curetting. It was histologically diagnosed to be a pituitary adenoma. The empty sella was elevated by coagulation of intrasellar dura and herniated arachnoid membrane, and then we filled the residual intrasellar cavity with bone fragments and fat. Postoperative hormonal examination showed normal findings, and MRI revealed obliteration of the empty sella. Surgical indication for primary empty sella is not established, but in cases associated with pituitary adenoma, transsphenoidal surgery is necessary. We reported a case of empty sella syndrome associated with GH secreting pituitary adenoma, and in this report, we introduced a new surgical technique for repairing an empty sella.
Adenoma↗
[Cockayne syndrome].
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Cockayne Syndrome↗
[Pallister-Hall syndrome].
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Anus, Imperforate↗
Two sisters with clinical diagnosis of Wiskott-Aldrich syndrome: is the condition in the family autosomal recessive?
We report two sisters in a family representing manifestations of Wiskott-Aldrich syndrome (WAS), an X-linked immunodeficiency disorder. An elder sister had suffered from recurrent infections, small thrombocytopenic petechiae, purpura, and eczema for 7 years. The younger sister had the same manifestations as the elder sister's for a 2-year period, and died of intracranial bleeding at age 2 years. All the laboratory data of the two patients were compatible with WAS, although they were females. Sialophorin analysis with the selective radioactive labeling method of this protein revealed that in the elder sister a 115-KD band that should be specific for sialophorin was reduced in quantity, and instead an additional 135-KD fragment was present as a main band. Polymerase chain reaction (PCR) analysis of the sialophorin gene and single-strand conformation polymorphism (SSCP) analysis of the PCR product demonstrated that there were no detectable size-change nor electrophoretic mobility change in the DNA from both patients. The results indicated that their sialophorin gene structure might be normal. Studies on the mother-daughter transmission of X chromosome using a pERT84-MaeIII polymorphic marker mapped at Xp21 and HPRT gene polymorphism at Xq26 suggested that each sister had inherited a different X chromosome from the mother. Two explanations are plausible for the occurrence of the WAS in our patients: the WAS in the patients is attributable to an autosomal gene mutation which may regulate the sialophorin gene expression through the WAS gene, or, alternatively, the condition in this family is an autosomal recessive disorder separated etiologically from the X-linked WAS.
Antigens, CD↗
The fate of human glial cells following transplantation in normal rodents and rodent models of neurodegenerative disease.
Investigations on xenografting in the brain have previously focused on the anatomical and functional integration of the transplanted neurons. More recently, astrocytes are being implicated as having complex functions following transplantation, and are being investigated to determine their role(s) in transplantation. The present study was undertaken to investigate the migration of human astrocytes following transplantation of thalamic, striatal, and mesencephalic tissue into the rodent striatum. Human donor fetuses (9-16 weeks in gestation) obtained through elective and spontaneous abortions were utilized in this study. Following transplantation, donor astrocytes were labeled with an antiserum directed against human glial fibrillary acidic protein. Our results demonstrate that astrocytic elements from all three tissue types are capable of incorporating into the host brain, and have a tendency to follow white matter tracts (such as the corpus callosum, internal capsule, and fiber bundles in the striatum). Human astrocytes, originating from the striatum and thalamus exhibited extensive migration, while migration was more limited in animals with ventral mesencephalon transplants. Ventral mesencephalon transplanted animal demonstrated positive astrocytes within the transplant, with processes (very few cell bodies) extending into white matter of adjacent host striatum. Astrocytes demonstrating immature morphology were observed with all transplant types, but were most prevalent in the striatal transplanted animals. The extent of astrocyte migration and the morphologies observed in this study reflect regional differences of the developing human brain. These results confirm and extend previous investigations on glial cell migration following transplantation in the brain.
Animals↗
Evolution of the noncoding regions in Drosophila mitochondrial DNA: two intergenic regions.
The sequences of the mitochondrial DNA (mtDNA) segment containing the two intergenic regions were determined for six species belonging to the Drosophila immigrans species group and compared to the corresponding segments of Drosophila species which had been studied previously. We found remarkable differences in the evolutionary rates of the two intergenic regions. The Intergenic I region, which lies between the tRNA(gln) and the tRNA(ile) genes, was found to be highly conserved in terms of both size (30 ntp) and nucleotide sequence among the species studied. In contrast, the sequences of the Intergenic II region, which lies between the tRNA(f-met) and the tRNA(ile) genes, showed considerable variation. The size of the Intergenic II region ranged from 0 to 88 ntp, and accurate alignment was possible only among sequences from geographical strains or very closely related species in the nasuta species subgroup. The observed differences in conservation of the two mtDNA intergenic regions are discussed in light of functional constraints on mtDNA sequences.
Animals↗
Development of human fetal ventral mesencephalic grafts in rats with 6-OHDA lesions of the nigrostriatal pathway.
Neuronal transplantation is an approach that can be exploited to study the development of the human central nervous system as well as being used in attempts to restore neurological function. In the present study, we have examined cellular events that appear to precede the development of dopamine nerve fiber extension by neurons from the human fetal ventral mesencephalon. These cellular events were examined using neuronal cell suspensions from human fetal ventral mesencephalic tissue (gestational ages 7-10 weeks) transplanted into the striatum of unilaterally lesioned 6-hydroxydopamine (6-OHDA) rats. Animals were sacrificed for immunohistochemistry 9-10 weeks after the transplantation prior to the manifestation of behavioral recovery. Histological analysis revealed tyrosine hydroxylase (TH) immunoreactive neurons in the grafts. The majority of these neurons had very short TH positive processes (60-70 microns), indicating that the maturation of grafted dopaminergic neurons was still incomplete. Immunostaining for the human specific intermediate neurofilament (hNF, clone: BF-10) showed dense neuronal fibers in the grafts. These fibers extended deeper into the host brain than the TH positive neuronal processes. The whole striatum, particularly the medial part of the striatum, exhibited long NF positive processes. Glial fibrillary acidic protein (GFAP) immunohistochemistry revealed fine astrocytic processes inside the grafts, which were clearly different from host reactive glial cells surrounding the grafts. These graft-derived glial processes tended to extend into the host brain deeper than the TH positive neuronal processes from the grafts. These early histological findings of the grafted human fetal ventral mesencephalon suggest that the graft-derived NF positive neuronal processes, as well as the glial processes, radiate from the grafted tissue and extend into the host brain prior to the extension of TH positive processes. These results further suggest that human-to-rat xenografts can be used to study the neural development of human fetal brain tissue.
Animals↗