[Function and plasma membrane of granulocytes].
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Aminopeptidases↗
Biomedical subjects
K Iwabuchi
Publications and source records attributed to K Iwabuchi.
[A new type of complicated form of hereditary spastic paraplegia showing mental deterioration, quadriplegia with muscular atrophy, sensory disturbance, extrapyramidal disorders, and epilepsy].
UNLABELLED: This study proposes a new type of complicated form of hereditary spastic paraplegia (HSP) and some problems on a clinico-pathological classification of HSP. The present study includes three male and two female patients from two families (A and B). In the family A, four siblings (two males and two females) were affected. Spastic paraplegia developed as an initial symptom. In the family B, a man was affected with spastic paraplegia which had started at the age of eleven. His two half-sisters are normal. All parents in two pedigrees are healthy. The parents in the family B are first cousins. CLINICAL FEATURES: Their physical development was normal, but all of them showed mild mental retardation. Gait disturbance due to spastic paraplegia and mental deterioration developed at the age of high teens. At the age of high 20's, they became unable to walk, because of progressive spastic paraplegia and other complicated neurological impairments including pyramidal disorders in the upper limbs, generalized neurogenic muscular atrophy, sensory disturbance and bradykinesia. Some cases showed rigidity and/or spasticity. At the age of high 30's, they became bed-ridden, because of quadriplegia with generalized muscular atrophy. Three cases in the family A suffered from convulsions which started at the high 30's. Mild athetoid movement in the face and neck was observed in three cases in the family A. All patients became apathetic and indifferent at least by the age of 40. Laboratory findings and data: CT scan yielded brain atrophy and dilatation of the lateral ventricles, atrophy of the corpus callosum and hyperostosis of the cranium.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
[Three cases of minocycline-induced pneumonitis].
All three patients complained of cough, fever and dyspnea. Their chest X-ray films revealed interstitial changes such as Kerley B lines. The results of lymphocyte stimulation tests were all negative for Minocycline (MINO), whereas the provocation tests were all positive in three cases. The onsets of symptoms appeared 7, 12 and 9 hours after administration of MINO respectively, which suggested type III allergy in terms of the latency period. In cases No. 1 and No. 3, lung tissue specimens obtained by transbronchial biopsy showed findings of mild acute eosinophilic pneumonia. The bronchoalveolar lavage fluid of case No. 3 showed eosinophilia. On diagnosing MINO-induced pneumonitis, the lymphocyte stimulation test is not always beneficial, whereas the provocation test is supposed to be a safe and sure method.
Adult↗
Platelet-derived neutrophil adherence-inhibiting factor in humans.
The effect of constituents of human platelets on leukocyte adherence was examined. Adherence-inhibiting factors (AIFs), which strongly inhibited neutrophil adherence to glass, were present in both cytosol and granule fractions of human platelets. On the Superose 6 gel chromatography (Pharmacia LKB Biotechnology, Uppsala, Sweden), the granular AIF was eluted as a single active peak (2,600 Kd), whereas cytosolic AIFs were eluted at two different positions (2,600 and 480 Kd). When platelets were stimulated by thrombin, granular AIF was released extracellularly without releasing a cytosolic marker. Using DE32 anion exchange chromatography and Superose 6 gel filtration, granular AIF was completely purified. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis analysis suggests that granular AIF consists of two subunits with molecular masses of approximately 340 and 190 Kd. Purified granular AIF inhibited human neutrophil adherence to glass, plastic, and type IV collagen-coated plastic, whereas it did not affect monocyte adherence. These results suggest that granular AIF inhibits neutrophil adherence not only via nonspecific adsorption sites, but also via type IV collagen receptors.
Animals↗
An autopsy case of peroneal muscular atrophy with rigidity and tremor. Ultrastructural and systematic morphometrical studies on peripheral nerves.
An autopsy case of hereditary peroneal muscular atrophy (PMA) with rigidity and static tremor is presented. The patient developed slowly progressive distal muscular atrophy of the legs at the age of 15 years. By the age of 52 years, PMA became marked associated with pes cavus, and tremor and rigidity of the extremities were noted. Motor and sensory conduction velocities gradually depressed and lost near the end of his life. At autopsy, the major neuropathological abnormalities involved the peripheral nervous systems, and were characterized by axonal atrophy and loss of myelinated fibers. These changes involved both the proximal and distal nerves, being more severely affected in the distal. The pathological changes in other regions of the nervous systems were mainly confined to the spinal cord, dorsal ganglia and spinal nerve roots, and pigmented neurons in the brain stem. Morphometrically, the total fascicular area was much smaller than in control, but the total number of myelinated fibers greatly outnumbered that of control 75,200 to 48,200 at the proximal sciatic nerve and then gradually decreased towards the periphery; however, even in the distal sural nerve, the total number of myelinated fibers exceeded that of control (6820 to 5469). Thus, the density of myelinated fibers were much higher, being 1.5 to 2 times greater, than in control. Its abrupt decline at the distal nerve might account for neurogenic atrophy of the distal musculature. Unmyelinated fibers were slightly increased in density and not atrophic. This case is unique in its clinicopathology and does not belong to any subtypes of PMA including "neuronal plus".
Adult↗
Sequential analysis of the thymocyte differentiation in fully allogeneic bone marrow chimera in mice. I. Relationship between functions and surface characteristics of thymocytes.
Differentiation of thymocytes according to surface phenotype, functional status and cell size was investigated using fully allogeneic bone marrow chimeras. Most of the donor-derived thymocytes obtained from chimeras 9 days after hematopoietic reconstitution were CD4-8- and IL2R+. At day 14, CD4+8+ cells became prominent in the thymus. Eighty-six per cent of thymocytes were CD4+8+ and 9% were CD4-8- at this stage. After day 21, the proportion of CD4+8- or CD4-8+ single positive cells transiently increased and then declined to normal level at day 42. Further, the mean size of CD4+ or CD8+ single positive cells in chimeric thymuses at day 21 after reconstitution was markedly larger than that at day 35. When proliferative responses to various stimuli (PMA + rIL2, anti-CD3 mAb (2C11) and anti-V beta 8 mAb (F23.1] were evaluated, significant responses were generated by thymocytes for the first time at around day 28 and the responses reached their peaks at day 35. These findings demonstrated that the process of thymocyte differentiation in the fully allogeneic chimeras was similar to ontogenic development as observed in fetal mice. However, the tempo at which the differentiation of surface phenotypes and development of functions proceeded was quite different from that seen in normal mice. The relationship among surface phenotypes, cell size and functions of developing thymocytes of bone marrow chimeras is discussed.
Animals↗
Sequential analysis of the thymocyte differentiation in fully allogeneic bone marrow chimera in mice. II. Further characterization of the CD4+ or CD8+ single positive thymocytes.
Differentiation of CD4+8- and CD4-8+ single-positive (SP) thymocytes in fully allogeneic bone marrow chimeras were investigated using multicolor cytometric analysis. The proportion of CD3+ cells in CD4+ SP population derived from donor mice considerably increased between day 12 and 14 after bone marrow transplantation (BMT), and gradually increased thereafter. The proportion of V beta 8+ cells in the CD3+CD4+ population remained constant (around 20%) at each period, suggesting that alpha and beta chains were used as TCR. The proportion of J11d+ cells in the CD4+ SP thymocytes transiently increased from day 12 to 14 and decreased thereafter, even though almost half of CD4+ SP cells were still dull J11d+ at day 35 after BMT. When CD8+ SP populations were analyzed, the proportion of CD3+ cells was very small until day 18. Thereafter, the proportion considerably increased and reached a maximum (83.2%) at day 21. The proportion of V beta 8+ cells in the CD3+ CD8+ SP population fell within range between 20 and 30%. However, before day 18, most of the V beta 8+ cells were dull positive, while after day 21 the majority were bright V beta 8+. Further, CD8+ SP cells at day 12, 14 and 18 were largely bright J11d+. After day 21, however, the proportion of bright J11d+ cells rapidly decreased. Similar results were obtained when the sequence of appearance of CD4+ and CD8+ SP cells was compared among bright CD3+, bright V beta 8+ or J11d- mature populations. The CD4+ SP cells regularly appeared earlier than CD8+ SP cells in the mature populations. These findings indicate that a considerable heterogeneity exists within both CD4+ and CD8+ SP populations and that the differentiation process for CD4+ SP cells precedes that for CD8+ SP cells.
Animals↗
Sequential analysis of distributions of donor-derived thymocytes bearing T-cell antigen receptor (TCR) and donor-derived Ia+ cells in thymuses of fully allogeneic bone marrow chimera in mice.
Lethally irradiated SJL/J mice were reconstituted with B10 bone marrow cells, and the process of thymic reconstitution by donor-derived cells positive for I-A or V beta 8 molecules was investigated. The donor-derived Ia+ cells appeared in the medulla on day 7 after reconstitution. The Ia+ cells became confluent up to day 14, and the cellularity in the medulla on day 17 was almost the same as that in the normal thymus. Dull V beta 8+ thymocytes were first recognized in the cortex on day 10 and were identifiable in the medulla by day 14. The V beta 8+ cells seemed to be mainly CD4+8+ double-positive. Furthermore, most of the V beta 8+ cells in the medulla of chimeras given cyclosporin A for 3 weeks after reconstitution appeared to be CD4+8+ thymocytes which bear a low concentration of TCR exist in the thymic medulla at a relatively early stage when donor-derived Ia+ cells have already settled there. The coincidental appearance and coexistence of Ia+ cells and TCR+ thymocytes in the medulla suggest that these histological characteristics may be related to the selection of thymocytes in this area.
Animals↗
[An autopsy case of complicated form of spastic paraplegia with amyotrophy, mental deficiency, sensory impairment, and parkinsonism].
An autopsied case of complicated form of spastic paraplegia with many unusual clinical and pathological features is reported. Present case: a 31-year-old male. His parents are first cousins. Pregnancy and delivery had been unremarkable. Though he was mentally retarded, his physical development was normal. He was considered normal until age 10. He suffered from progressive disturbance in gait at the age of 11. He could not walk without assistance at the age of 22. Neurological examination revealed the following findings. He was obese and mentally deteriorated. Spastic paraplegia with increased tendon reflexes and pathological reflexes was prominent. Though slight sensory disturbance was present in the lower extremities, neither involuntary movement nor cerebellar ataxia was observed. In the age of late 20's, dementia, general muscular atrophy, and Parkinsonism developed. At the age of 30, he could not move by himself. He was apathic and indifferent, and showed forced laughing. Muscle tonus was flaccid because of general muscular atrophy and peripheral neuropathy. He died of acute gastric enlargement. Neuropathological findings were characterized by mal-development of the central nervous system (CNS) and the multisystem degeneration. There existed cerebral white matter hypoplasia with hypogenesis of the corpus callosum and ectopia of neurons of the cerebral and cerebellar cortex. Hypoplasia of melanin pigment was also observed in the remaining neurons of the substantia nigra and the locus ceruleus. Many neurons in the CNS included lipofuscin granules of variable shapes. Some of them showed clusters of several block-like inclusions which were green with luxol fast blue and cresyl violet stain.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
[An autopsied case of idiopathic late cortical cerebellar atrophy--comparison with other cortical cerebellar atrophy].
A 68-year-old man without familial history developed ataxic gait and sensory disturbance in the lower extremities. At the age of 74, neurological examination revealed marked cerebellar ataxia of all limbs, dysarthria, sensory disturbance of glove and stocking type in the extremities, and slight neurogenic muscular atrophy. There were no mental deterioration and dysautonomia. He died of pneumonia at the age of 74. Neuropathological findings. The cerebellum was decreased in size. Microscopically, there were severe disappearance of Purkinje cells in the cerebellar vermis and hemispheres. The molecular layer, granular cell layer, and cerebellar white matter were preserved. Neurons of the inferior olivary nuclei were also spared. In the spinal cord, there was myelin pallor in the posterior column predominant in Goll's fascicule and moderate atrophy of neurons in the anterior horn. Degeneration of the posterior roots was greater than that of the anterior roots. No abnormal findings were found in the extrapyramidal system, cranial nerves, and cerebrum. We compared this case clinicopathologically with other diseases with cortical cerebellar atrophy; alcoholic cerebellar degeneration, phenytoin intoxication, neuroleptic malignant syndrome, and subacute paraneoplastic cerebellar degeneration. In conclusion, idiopathic late cerebellar cortical atrophy (LCCA) was different clinicopathologically from the other diseases. Especially, LCCA showed the characteristic topography of Purkinje cells loss sparing the molecular and granule cell layers.
Aged↗
[An autopsied case of alcoholic cerebellar degeneration with spastic paraplegia and neuropathy].
A 45 year-old-female was admitted to Kanagawa Rehabilitation Center because of marked spastic paraplegia. There was no family history of neurological diseases. She had been drinking a great excess of alcohol since twenty years of age. Though she noticed the unsteadiness of gait several years prior to the admission, she had not been examined. On admission neurological examination revealed pyramidal weakness of both legs, and slight sensory impairment in distal part of the lower extremities. But she had neither difficulty in speech nor abnormal findings in the upper extremities. Her mentality was well preserved. Though computed tomography revealed cerebellar cortical atrophy, no signs of cerebellar impairment could not be found except spastic paraplegia. Blood chemistry failed to reveal liver dysfunction. There was no pernicious anemia. These symptoms were almost stationary until her death. At the age of 53, she died of gastric cancer. Postmortem examination disclosed marked degeneration of cerebellum and spinal cord. Cerebellar cortical degeneration, which was characterized by involvement of all layers of the cortex, showed unique distribution. The lingula, central lobule, culmen, superior portion of declive, anterior lobules and anteromedial half of simple lobule were severely degenerated, while other lobules were spared excluding moderate degeneration in inferomedian portion of the hemisphere. These cortical degeneration was prominent much more in vermis than in hemisphere. There was a mild myelin pallor in the lamellar white matter and slight fibrous gliosis. In the dentate nucleus, there were moderate neuronal loss with gliosis.(ABSTRACT TRUNCATED AT 250 WORDS)
Alcoholism↗
[Pulmonary alveolar proteinosis].
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Humans↗
Purification and partial characterization of platelet-derived adherence-inhibiting factor in guinea pig.
Purification and partial characterization of adherence-inhibiting factor (AIF) of platelet-granule fraction in guinea pig were studied. When freshly prepared platelet-granule fraction was subjected to a gel filtration, two neutrophil adherence-inhibiting peaks, designated AIF-I (2,800 kDa) and AIF-II (12 kDa), appeared. AIF-I was sensitive to diisopropylfluorophosphate (DFP) and originated from lysosomes, whereas AIF-II was insensitive to DEP and localized in alpha-granules. Both AIFs were released from platelets by a thrombin stimulation. As the total activity of AIF-I was about 5-fold higher than that of AIF-II, AIF-I was purified and characterized. When purified AIF-I was analyzed on SDS-polyacrylamide gel electrophoresis, the 340 kDa protein band and the other large protein band were observed. Under reducing condition, AIF-I was separated into three components (340, 190 and 165 kDa). AIF-I significantly inhibited neutrophil adherence to artificial substrata and to type IV collagen-coated plastic surface, but not to fibronectin- or plasma-coated plastic surfaces, suggesting that AIF-I inhibits neutrophil adherence not only via nonspecific adsorption sites but also via type IV collagen receptors.
Animals↗
Further observation of Japanese Creutzfeldt-Jacob disease with widespread amyloid plaques.
An autopsy case of Creutzfeld-Jacob disease with widespread amyloid plaques is reported. A 45-year-old Japanese man, whose father had died of a similar disease, had a 5-year illness characterized by progressive cerebellar signs. Mental changes and brain-stem signs developed in the late stage. Myoclonus frequently occurred. Akinetic mutism ensued. The autopsy revealed spongiform encephalopathy with widespread amyloid plaques and extensive degeneration of the white matter. This disease, Western Gerstmann-Sträussler-Scheinker disease and panencephalopathic type of Creutzfeld-Jacob disease are discussed.
Amyloid↗
Mucoepidermoid carcinoma arising in a lymphoepithelial cyst--report of a case and review of the literature on branchiogenic carcinoma.
A 59-year-old Japanese man who had recently noticed a gradual enlargement in a nodule located in the left anterior part of his neck was admitted for surgical treatment. The histopathological features of the resected tumor were consistent with a typical lymphoepithelial cyst, however, the inner surface epithelium of the cyst showed histological transition to mucoepidermoid carcinoma, which was partially invading the cyst wall. Despite clinical efforts in a systemic search, no primary lesions which could have metastasized were found. The patient hasn't shown any evidence of recurrence since his tumor resection over 2 years ago. Besides this rare case report, a review of the English literature reveals 22 cases of possible branchiogenic carcinoma over the last 10 years, including 21 cases whose histological features were of squamous cell carcinoma and 1 whose histological features revealed mucoepidermoid carcinoma. The present case is thus only the second proven case of low-grade mucoepidermoid carcinoma arising within a branchiogenic cyst.
Branchioma↗
"Grumose degeneration" of Trétiakoff.
We reviewed Trétiakoff's "grumose degeneration (GD)" which described pathologic cellular change in the substantia nigra (SN). This term has been occasionally used up to the mid-1960s by Greenfield et al.; it has rarely been used after the 1970s. This study emphasises the following: (1) after the 1970s, GD has sometimes been signalled in the SN under various names, such as "spheroid with foamy appearance", "granular spheroid", "saccular structure", or "foamy spheroid body"; (2) the ultrastructure of GD is unknown, being entirely different from that of typical axonal swellings (so-called "spheroids"); (3) more attention should be paid to GD in the SN because its nature has remained unclarified since the original description; and (4) "GD" in the cerebellar dentate nucleus is essentially different from Trétiakoff's GD.
Cerebellar Nuclei↗
Donor and recipient specific tolerance in cells from semi-allogeneic, H-2 subregion compatible or fully allogeneic bone marrow chimeras attributable to clonal deletion.
Specificities of tolerance induced in allogeneic bone marrow (BM) chimeras which had been established by injecting allogeneic BM cells pretreated with anti-Thy-1 mAb alone (without complement (C)) were analyzed using Simonsen's splenomegaly assay. Lymphocytes from fully allogeneic, semi-allogeneic and H-2 subregion compatible BM chimeras were specifically unresponsive to donor and recipient antigens (Ag). However, cells from H-2 subregion compatible chimeras initiated as vigorously a GVHR in F1 recipient mice, which were disparate at H-2K and I-A regions, as did spleen cells of donor mice, which were incompatible at the entire H-2 and minor histocompatibility regions of the recipients. The donor cells from such chimeras that initiated these considerable GVHR were either CD4+ or CD8+ T cells. Furthermore, synergistic effects by the CD4+ and CD8+ T lymphocytes were also observed. We found no evidence for a suppressive mechanism(s) in maintenance of the specific tolerance in allogeneic chimeras. Further, when lymphoid cells from these chimeras were adoptively transferred to irradiated mice of the donor strain and maintained for 5 days in the absence of recipient Ag (tolerogen), the adoptively transferred cells were shown to retain their unresponsiveness to the recipient Ag. These results reveal that T lymphocytes from allogeneic BM chimeras prepared by our method had been specifically induced to a tolerant state to both donor and recipient Ag and that the major mechanism of induction and maintenance of long-lasting tolerance is attributable to clonal deletion of both CD4+ and CD8+ T cell subsets rather than to the development of a population of suppressor cells of any sort.
Animals↗
Positive selection of a T-cell subpopulation in the thymus in which it develops.
In SWR mice the expression with high-density V beta 17a (high V beta 17a) of the T-cell antigen receptors correlates with the CD4+8- subpopulation of thymocytes. By contrast, in thymocytes of SJL mice the expression of high V beta 17a is observed on the CD4+8- or CD4-8+ subpopulation. However, when the thymocytes from SWR mice have been developed in the SJL or B10 thymus but not in the H-2 compatible DBA/1 thymus, a greater proportion of thymocytes that express high V beta 17a was found to be CD4-8+. By contrast, only a small proportion of KJ23a+ thymocytes from SJL mice that had differentiated in the thymus of SWR or DBA/1 mice was CD4-8+, whereas a high proportion of CD4+8- cells expressed V beta 17a. Further, an intermediate proportion of KJ23a+ thymocytes that had derived from SJL donor mice was present on CD4-8+ thymocytes that had developed in B10.A(4R) thymus. These findings demonstrate that the appearance of a particular subpopulation of thymocytes (CD4-8+ with a beta chain of T-cell antigen receptor identified as V beta 17a) is determined by the histocompatibility complex products that are expressed in the thymic microenvironment in which the T cells develop.
Animals↗