MORPHOLOGICAL AND BIOCHEMICAL STUDIES OF A CASE OF MUCOPOLYSACCHARIDOSIS II (HUNTER'S SYNDROME)

An autopsy case of a 19-year-old boy who had shown typical gargoyle features, strictly consistent with mucopolysaccharidosis type II (Hunter's syndrome) was reported. Histologically, cytoplasmic vacuolar change was found In hepatocytes, sinusoidal epithelium of spleen, follicular cells of thyroid, Sertoli cells of testis, chromophobe cell of pituitary and generalized fibroblast-like cells including meninges, cardiac valve and periosteum. The vacuoles consisting of membrane-bound structures with flocculus protein-like material and occasional electron dense bodies on electron microscopy, were considered to be the site of mucopolysaccharide deposition by histochemical analysis. Deposition of lipid material consistent with so-called membranous cytoplasmic body was observed in the neurons of central, peripheral and autonomic nervous system. Hepatosplenomegaly could be explained by cytoplasmic deposition, but the cause of cardiomegaly remained further to be studied. Biochemically hepatic mucopolysaccharide was identified as heparan sulfate, while in the kidney dermatan sulfate and heparan sulfate were detected. The correlation between morphology and biochemistry, and between deposition and degeneration was discussed.     

Slight difference in primary amino acid sequence of p17 matrix protein of HIV-1 exerts profound influence on its antigenicity

Summary.  HIV-1 p17 antigen has been studied for its biological significance in vitro as well as its immunological roles in vivo. By immunological approach of antibody-binding to HIV-1 p17 antigens of several subtypes in combination with computerized analysis of those tertial structures, it became evident that, irrelevant of similarity of linear amino acid sequence of different HIV-1 subtypes, a few amino acid substitutions close to or distant from specified epitope(s) affected their tertial structure resulting in change in ability of its binding to selected antibody. ELISA employing two monoclonal antibodies, A144 and C415, could detect p17 of subtypes A and B, but not of subtypes C, D, and E. Since the epitope site corresponding to A144 has been reported to be important for biological activity of p17 of HIV-1, change in tertial structure around this epitope may explain some difference in biology of HIV-1, such as infectivity of subtypes B and E. Accepted January 9, 1998 Received October 24, 1997     

Neurofilaments of Kultschitsky cells in human lung

The immunohistochemical localization of three triplet proteins of neurofilaments in normal Kultschitsky cells and tumourlets of the human lung has been studied using avidin-biotin-peroxidase complex (ABC) method. Kultschitsky cells and tumourlets have been stained with antisera against 68 K, 150 K and 200 K dalton components of the neurofilaments, respectively. Ultrastructural observations of human Kultschitsky cells have revealed the presence of bundles of intermediate filaments as well as microtubules and neurosecretory-type granules. In the tumourlets, similarly sized filaments were found, but were relatively scarce. Since intermediate filaments are thought to be specific to differentiated cells and neurofilament proteins are restricted to the neuronal tissues, we conclude that Kultschitsky cells of the lung are of neuronal nature.     

Numerous mast cells in an 11-deoxycorticosterone-producing adrenocortical tumor. Histologic evaluation of benignancy and comparison with mast cell distribution in adrenal glands and neoplastic counterparts of 67 surgical specimens

Pathologic study of a rare 11-deoxycorticosterone-producing adrenocortical tumor causing primary aldosteronismlike signs and symptoms, revealed several characteristic features as follows: (1) fairly large size with histologic features corresponding to those of benign zone glomerulosa-type aldosteronoma, (2) lack of spironolactone (S) bodies despite S administration, and (3) heavy mast cell infiltration. In order to explain this rare histology, the localization of mast cells in the adrenal glands and functioning adrenocortical tumors of 67 surgical specimens were investigated. The results of the study supported the view that detection of mast cells helps in the differentiation of mineralocorticoid-producing tumors from cortisol-producing ones, and that the observed mast cell infiltration was due, in part, to its production of 11-deoxycorticosterone.     

Mesp1-nonexpressing cells contribute to the ventricular cardiac conduction system.

Previous fate mapping analysis, using Cre recombinase driven by the Mesp1 locus, revealed that Mesp1 is expressed in almost all of the precursors of the cardiovascular system, including the endothelium, endocardium, myocardium, and epicardium. Mesp1-nonexpressing cells were found to be restricted to the outflow tract cushion and along the interventricular septum (IVS), which is a location that is suggestive of specialized cardiac conduction system (CCS). In our current study, we examined the identity of these IVS cells by using the pattern of beta-galactosidase activity in CCS-lacZ mice. In addition, by crossing Mesp1-Cre and floxed GFP reporter mice with CCS-lacZ mice, we have calculated that approximately 20% of the ventricular CCS within the IVS corresponds to Mesp1-nonexpressing cells. These data suggest that the ventricular CCS is of heterocellular origin. Furthermore, we indicate a possibility that a population of the cells that contribute to the ventricular CCS might be distinguished at an early stage of development.     

The ophthalmic artery and its branches,measurements and clinical importance

Summary Seventy-one Caucasian orbits (36 right, 35 left) were studied by dissection. The diameter of the ophthalmic a. (2 mm from the origin) was 1.54 ± 0.04 mm (male) and 1.31 ± 0.05 mm (female). In individual cases, there were no significant differences in vessel diameter between the right and left sides but, differences in vessel diameter between males and females were more commonly observed in the arteries which leave the orbit (extraorbital group), the individual vessels having a larger diameter in males. The incidence of the ophthalmic a. passing in the orbit medially under the optic n. was 18.6%. The lacrimal a. was observed to arise from the ophthalmic a. in only 82.5% of the cases examined, 15.9% of the cases showed the origin to be at the anastomotic branch of the middle meningealThis article is dedicated to Pr Dr Hoepke on occasion of his 100th birthday     

The development of extranodal lymphoid follicles in experimental bronchopneumonia.

Extranodal formation of lymphoid follicles was morphologically studied in experimental bronchopneumonia. Control gnotobiotic mice had no peribronchial lymphoid follicles and only lymphatic vessels were traced from the terminal bronchiolar region toward larger bronchi. During the week after intranasal inoculation of mycoplasma pulmonis, lymphoid follicles developed in the terminal portion of the lymphatics by the accumulation of small lymphocytes. A loose network of mesenchymal cells and early infiltration of macrophages, following stromal edema, seemed to play an important role in the early accumulation of lymphocytes. Blastic transformation was seen frequently in the center of the accumulated lymphocytes. Two weeks after inoculation plasma cells emerged conspicuously in the periphery of the lymphoid follicles, and the acute phase of bronchopneumonia began to subside. Typical germinal centers with tingible body macrophages and dendritic reticulum cells developed when the bronchitis persisted in a chronical manner.     

The molecular structures of 1,1-diphenylethyl methacrylate and triphenylmethyl methacrylate

The molecular structures of 1,1-diphenylethyl methacrylate (1,1-DPEMA) and triphenylmethyl methacrylate (TrMA) were determined by means of X-ray diffraction. 1,1-DPEMA: monoclinic, space group P2₁/a,a = 9,666(6), b = 19,94(2), c = 8,132(6) Å, β = 104,49(7)°, and Z = 4; TrMA: monoclinic, space group P2₁/n, a = 17,349(3), b = 9,487(2), c = 11,254(2) Å, β = 102,30(2)°, and Z = 4. Both structures were solved by the direct method and refined by the block-diagonal least-squares procedure to R = 0,175 and 0,056 for non-zero reflections, respectively. In both molecules, conformations about the C(1)? C(2) and C(1)? O(1) bonds are all synperiplanar and one of the two or three phenyl groups attached to the C(5) atom is in trans to the O(2).