[Changes in the organization of the intermediate filament system of human fibroblasts in lysosomal storage diseases and their modeling].
The organization of the system of the vimentin intermediate filaments (IFs) in human fibroblasts in lysosomal storage diseases (Fabry's disease, mannosidosis) and their modelling has been studied in vitro. It was shown that during accumulation of nonhydrolyzable compounds, hypertrophy of the lysosomal compartment is accompanied by formation of ring-shaped bundles IFs, surrounding apparently these increased organelles. The changed organization of IFs is characteristic of polarised pathological cells in monolayer, and after repassage it is retained only at the spreading state; on transition from the discoid to extended cellular form there occurred the centrifugal shift of ring-shaped structures of IFs to active cell border and gradual restoration of radial fibrillar state of IFs. It is suggested that on intralysosomal storage of unsplit compounds reorganization of the vimentin-type IFs in ring-shaped structure is necessary for optimal distribution and stabilization into the cytoplasm of large amounts of increased lysosomes with exo- and endogenous contents. In condition of free spreading (i. e. with diminished cell density) the restoration of normal fibrillar IF organization may be due to the loss of considerable number of hypertrophied lysosomes; the involvement of lysosomal membrane in formation of active cellular border is not to be ruled out.
Ivleva
T S
TS
Tint
I S
IS
Bershadskiĭ
A D
AD
Vidershaĭn
G Ia
GIa
D002478:Cells, Cultured; D000795:Fabry Disease / Q000473:pathology; D005347:Fibroblasts / Q000648:ultrastructure*; D006801:Humans; D007382:Intermediate Filaments / Q000648:ultrastructure*; D016464:Lysosomal Storage Diseases / Q000473:pathology*; D008247:Lysosomes / Q000648:ultrastructure; D008856:Microscopy, Fluorescence; D015388:Organelles / Q000648:ultrastructure; D012867:Skin / Q000648:ultrastructure; D008363:alpha-Mannosidosis / Q000473:pathology
Izmenenie organizatsii sistemy promezhutochnykh filamentov v fibroblastakh cheloveka pri lizosomnykh bolezniakh nakopleniia i ikh modelirovanii.