0000000000235582
AUTHOR
K. H. Langer
Lecithin-cholesterol-acyltransferase deficiency: autosomal recessive transmission in a large kindred.
Thirty-four members of a single Sardinian kindred with lecithin-cholesterol-acyltransferase deficiency have been studied. The kindred spans four generations and the parents of the two affected siblings are blood relatives. Segregation of the acyltransferase deficiency gene in the family clearly demonstrated an autosomal recessive mode of inheritance. Thirteen family members, including all obligate heterozygotes, had roughly half-normal acyltransferase activities (mean +/- S.D. = 0.39 +/- 0.06 mU/ml) when compared to 17 intrafamilial controls and spouses (mean +/- S.D. = 0.72 +/- 0.09 mU/ml) and 40 blood donors from Marburg/Lahn (mean +/- S.D. =0.76 +/- 0.1 mU/ml). Characterization of the he…
Characterization of cells involved in the formation of granuloma. An ultrastructural study on macrophages, epitheloid cells, and giant cells in experimental tubulo-interstitial nephritis.
In experimental tubulo-interstitial (anti-basement membrane) nephritis of the rat, granulomatous inflammation develops around immunologically altered tubular basement membranes. The present light- and electron microscopic studies indicate that in the course of the granulomatous reaction, tissue monocytes evolve from blood monocytes and pursue two independent pathways of differentation. On the one hand they may differentiate into macrophages ("distant from tubules") or, alternatively, into epitheloid cells ("adjacent to tubules"). The latter, through cell fusion, develop into multinucleated giant cells of the Langhans' type. The cytoplasmic components of the epitheloid cells and the multinuc…
Cell fusion as a mechanism for the formation of giant cells (Langhans’ type)
The formation of multinuclear giant cells of the Langhans' type in tubulo-interstitial auto-immune nephritis in the rat has been investigated by means of autoradiography. While in the majority of giant cells all nuclei were radiolabeled, in a few both labeled and unlabeled nuclei were present. This latter finding represents strong evidence in favour of the hypothesis that giant cells do not form by endomitotic processes but rather through fusion of certain precursor cells. According to previous studies this precursor cell population consists mainly of epitheloid cells, i.e. modified monocytes.
Endocytotic activity in epitheloid and Langhans’ giant cells
In the experimental tubulo-interstitial (anti-basement membrane) nephritis in the rat, electron microscopic studies after the in vivo microinjection of native ferritin in areas of granulomatous inflammation near the surface of the kidney indicate that epitheloid and multinucleate Langhans’ giant cells are capable of endocytosis and particularly of micropinocytosis. This suggests the possibility that endocytotic activities as well as secretion phenomena are important in the immune defense mechanisms linked with these “specifically” developed cells.
Biophysikochemische Strukturen des glomerulären Filters
Die Feinstrukturen sowie die biophysikalischen und biochemischen Eigenschaften des glomerularen Filters fur die Permeation von Makromolekulen werden dargestellt. Unter Berucksichtigung der unterschiedlich lokalisierten Immunkomplexablagerungen bei den verschiedenen Formen der Glomerulonephritis des Menschen orientieren sich Beschreibung und Diskussion an bestimmte Schichten des Filters: 1. endothelial — subendothelial (=porenhaltiges Endothel und lamina rara interna der Basalmembran), 2. membranos (=Lamina densa der Basalmembran) und 3. subepithelial — epithelial (=Lamina rara externa der Basalmembran und Podozyten mit Fusfortsatzen und Schlitzmembranen). Dabei wird hervorgehoben, das die g…