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Biomedical subjects

T Kajii

Publications and source records attributed to T Kajii.

At least 91 records · Page 5Linked to original sources

Neuroaxonal dystrophy of neonatal onset with unusual clinicopathological findings.

A male infant with neuroaxonal dystrophy of neonatal onset is described. He was hypertonic, and exhibited horizontal nystagmus, occasional tremulous movements of the head and extremities, and an absence of waves III through V on auditory brain stem response examination. An autopsy at age 11 months revealed spheroid formation that was predominant in the cerebellum and brain stem, and dysmyelination that was mainly seen in the white matter of the cerebellum. This is in contrast with most of the previously reported patients of infantile neuroaxonal dystrophy in which these changes were evenly distributed throughout the central, peripheral and autonomic nervous systems.

Axons↗

Familial supernumerary non-satellited microchromosome.

A small, non-satellited and metacentric supernumerary chromosome was ascertained in an amniotic fluid cell culture. The supernumerary chromosome was present in four of seven family members karyotyped. Of these four carriers, two were phenotypically normal, while the other two, the fetus and its elder sister, suffered from methylmalonic acidemia. The supernumerary chromosome was apparently stable, being transmitted through three generations without causing mosaicism. It had a single C band, seven G bands and was silver staining negative, a hitherto undescribed situation.

Adult↗

Neutrophil adhesion abnormality with deficient surface membrane proteins (gp 110 and p 98): the effect of their antibodies on the function of normal neutrophils.

A sister and brother previously described with neutrophil adhesion defects and a lack of two neutrophil membrane proteins, glycoprotein with a molecular weight of 110 K and 115 K, were further studied. Rabbit polyclonal IgG antibodies were raised against neutrophil membrane proteins of approximately 110 K from normal individuals, and absorbed with the siblings' neutrophil membrane proteins. The antibodies thus absorbed reacted with two normal neutrophil membrane proteins, a 110 K glycoprotein and a 98 K protein, which were totally deficient in the siblings' neutrophils. The polyclonal antibodies were also reactive with a 95 K membrane protein of normal lymphocytes. The protein was missing in the siblings' lymphocytes. Three membrane proteins, Mac-1, LFA-1, and p 150, 95 each consisting of a specific alpha-subunit and a beta-subunit common to them, have been reported to be deficient in neutrophils from several patients with a neutrophil adhesion disease. Using monoclonal antibodies to Mac-1 alpha, LFA-1 alpha, and to the beta-subunit, it was deduced that the siblings' neutrophils lacked the above three membrane proteins. The disease in the siblings is thus identical with that previously described. Sodium dodecyl sulfate polyacrylamide gel electrophoresis of immunoprecipitates, formed by reacting labeled normal neutrophil membrane proteins with our polyclonal antibodies, revealed three bands, almost identical with that of immunoprecipitates formed with the anti-beta-subunit monoclonal antibody. This finding indicates that the polyclonal antibodies reacted mainly with the beta-subunit of the membrane proteins.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Detection and isolation of an acquired immune deficiency syndrome (AIDS)-related virus (HTLV-III/LAV) from a Japanese boy with AIDS-related complex.

We attempted to isolate acquired immune deficiency syndrome (AIDS) virus from a Japanese hemophiliac with AIDS-related complex (ARC). After cocultivation of leukocytes from his peripheral blood with those of a healthy adult, reverse transcriptase activity and AIDS viral antigens were detected by immunofluorescence and radio-immunoprecipitation methods, respectively. Moreover, an electron microscopic study revealed the presence of viral particles consistent with AIDS virus. These retroviruses were further propagated. We designated the first Japanese isolate of AIDS virus as YU-1.

Acquired Immunodeficiency Syndrome↗

Narrow host range of AIDS-related retroviruses (YU-1, 2, 3, 4) isolated from Japanese hemophiliacs: inability to infect H9, Molt-4, and MT-4 cells.

Infectivity was assayed by infecting human T-lymphocytes, H9, Molt-4, and MT-4 cells with different strains of AIDS viruses (HTLV-III, LAV, ARV, and YU viruses). Human T-lymphotropic virus type-III (HTLV-III), lymphadenopathy-associated virus (LAV), and AIDS-associated retrovirus (ARV) were able to infect all kinds of target cells tested and to induce virus-specific antigens, whereas Japanese isolates, YU viruses, infected only interleukin-2-dependent human T-lymphocytes. Long-term propagation of the YU viruses and pretreatment of the cells with Polybrene did not allow the YU virus to produce viral antigens by infecting H9, Molt-4, and MT-4 cells. Thus, YU viruses have a rather narrow host range as compared with HTLV-III, LAV, and ARV. These different infectivities may be reflected in the progress and symptoms of AIDS in vivo.

Acquired Immunodeficiency Syndrome↗

Purification and characterization of esterases D1 and D2 from human erythrocytes. Evidence that they are monomers.

Esterase D1 and esterase D2, two common esterase D (EC 3.1.1.1) isozymes, were isolated and purified from human erythrocytes. Their substrate specificity, pH profile and Km values were essentially identical. Their molecular mass was the same at 34 kDa on sodium dodecyl sulfate/polyacrylamide electrophoresis and at 27 kDa on Sephadex G-100 gel filtration. Antisera to each of the esterase D1 and esterase D2 isozymes were successfully raised in chickens; each antiserum reacted identically with both isozymes. These findings indicate that the isozymes are close to each other in structure. The fact that the molecular mass of the esterase D1 and esterase D2 isozymes computed on sodium dodecyl sulfate/polyacrylamide electrophoresis was close to that obtained on Sephadex G-100 gel filtration in non-dissociating buffer indicates that the isozymes are not dimers bound by disulfide bonds or a noncovalent force. These facts together indicate that the esterase D isozymes are monomers, contrary to the prevailing view that they are dimers.

Amino Acid Sequence↗

Ontogenic studies on the appearance of two classes of immunoglobulin-forming cells in the spleen of the Aleutian skate, Bathyraja aleutica, a cartilaginous fish.

We identified the presence in the Aleutian skate, Bathyraja aleutica, of two classes of immunoglobulins (Ig), a high molecular weight Ig analogous to mammalian IgM and a low molecular weight Ig, similarly to the spiny rasp skate, Raja kenojei, (Kobayashi, K. et al., Mol. Immunol. 1984. 21: 397), using an immunological cross-reaction with the specific antisera to the spiny rasp skate Ig components. The antigenic similarity of the heavy chains of the Ig of the Aleutian skate and those of the spiny rasp skate was less than that between their light chains. Two types of Ig-producing cells, one producing the high molecular weight and the other forming the low molecular weight Ig, were present in the spleen of embryos and adults in the Aleutian skate at a ratio of 5-6:4-5. The number of these Ig-producing cells increased with advancing development of the embryos but was 1/20 to 1/50 of those of adults. Cells, each of which were capable of forming both classes of Ig, were found in the spleen of embryos but not in that of adults. These results suggested that the spleen of the Aleutian skate is the primary lymphoid organ for B lymphocyte differentiation and proliferation, possibly equivalent to the bursa of birds.

Animals↗

Replication of X chromosomes in complete moles.

DNA replication patterns of X chromosomes in complete hydatidiform moles were studied using cultured fibroblasts from three 46,XX moles resulting from duplication of a haploid sperm, and from a 46,XY mole originating from dispermy. Control cultures included skin fibroblasts from an adult woman and a female fetus as well as PB lymphocytes from an adult woman. Cultures were treated with 5-bromo-deoxyuridine for the last 2-4 h of the S phase, and the chromosome slides prepared were stained by the Hoechst 33258-Giemsa procedure. Each of the three XX moles studied revealed one early-replicating and one late-replicating X chromosomes, while the XY mole revealed one early-replicating X chromosomes. DNA replication patterns of molar X chromosomes were similar to those of adult and fetal fibroblasts, but different from those in adult lymphocytes. These findings indicate that DNA replication kinetics of molar fibroblasts are tissue-specific rather than origin- or developmental-stage specific.

Cells, Cultured↗

J-chain-like component in 18-S immunoglobulin of the skate Raja kenojei, a cartilaginous fish.

A J-chain was found in the IgM, an 18-S pentameric immunoglobulin, of the skate, Raja kenojei, a cartilaginous fish, by means of gel filtration-column chromatography of reduced and radioalkylated immunoglobulin, followed by alkaline-urea polyacrylamide gel electrophoresis. The J-chain showed an electrophoretic mobility equivalent to that of the light chain, and much slower than that of the J-chain in human IgM. Its electrophoretic banding patterns, however, were different from those of the light chain. Its mol. wt of 17,500 was close to that of mammalian J-chain. The J-chain was not found in another immunoglobulin of the skate, a 9-S dimer held by a noncovalent force. No immunological cross-reactivity was observed between the skate J-chain and human and chicken J-chains. In view of these findings, it was concluded that the J-chain in the skate IgM is both electrophoretically and antigenically considerably different from the J-chain in mammalian IgM.

Animals↗

Supraumbilical midabdominal raphe and facial cavernous hemangiomas.

A 7-week-old Japanese female infant with supraumbilical midabdominal raphe and cavernous hemangiomas of the face is described. The combination of malformations in the infant, together with that in two infants previously reported from Germany, constitutes a clinical entity.

Abdomen↗

Trismus-pseudocamptodactyly syndrome in a Japanese family.

A family in which five individuals through three generations were affected with trismus-pseudocamptodactyly syndrome is reported. The propositus, an 8-year-old boy, had a right embedded ear, inability to open the mouth fully, pseudocamptodactyly and soft tissue syndactyly of the toes. His paternal grandmother, father, paternal uncle and his paternal cousin had pseudocamptodactyly. The occurrence of the syndrome in this Japanese family indicates that the syndrome is not exclusively of Dutch origin.

Adult↗

Double precipitin arcs of IgA myeloma sera on immunoelectrophoresis. Origin and suggestion of IgA2m(2) allotype.

The serum from two patients with IgA myeloma displayed double precipitin arcs upon immunoelectrophoresis with class-specific (light chain absorbed) anti-IgA antisera obtained by immunization with monoclonal IgA1, but not IgA2 proteins. Both sera contained large amounts of monoclonal IgA2m(2) together with some polyclonal IgA1. No incomplete IgA molecules were found. The double precipitin arcs resulted from the reaction of large amounts of monoclonal IgA2m(2) with class-specific antibodies, and of small amounts of polyclonal IgA1 with subclass-specific anti-IgA1 antibodies of the same antiserum. Such double arcs, if not due to incomplete IgA molecules, could indicate the IgA2m(2) nature of the monoclonal IgA in these rare sera.

Aged↗

Translocation (X;13)(p11.21;q12.3) in a girl with incontinentia pigmenti and bilateral retinoblastoma.

A de novo t(X;13)(p11.21;q12.3) translocation is described in an 19-month-old girl with incontinentia pigmenti (IP) and bilateral retinoblastoma. Based on previously reported two girls and this patient, each with a structural X chromosome abnormality and IP, it was assumed that the locus for IP is at Xp11.21. Q-banding analysis revealed that the translocated chromosomes were of paternal origin. The derivative X chromosome was late-replicating in 9% of cultured peripheral blood lymphocytes and in 1% of skin fibroblasts. The erythrocyte esterase D activity in the patient was normal. Several possibilities were considered for possible causative relationship between the X/13 translocation and the development of retinoblastoma. One possibility involved functional monosomy of 13q14 in a minority of retinoblasts due to the spreading of inactivation of the translocated X chromosome segment.

Chromosome Banding↗