A comparative study of the lesions in cultured fibroblasts of humans and four species of animals with Chediak-Higashi syndrome

1987 ◽  
Vol 28 (2) ◽  
pp. 445-454 ◽  
Author(s):  
Jocelyn D. Penner ◽  
David J. Prieur ◽  
John M. Opitz ◽  
James F. Reynolds
1986 ◽  
Vol 238 (2) ◽  
pp. 589-595 ◽  
Author(s):  
A L Miller ◽  
R Stein ◽  
M Sundsmo ◽  
R Y Yeh

Chediak-Higashi-syndrome cultured skin fibroblasts were used to study the possible involvement of lysosomal enzymes and lysosomal dysfunction in this disorder. Our evidence indicated that Chediak-Higashi fibroblasts displayed a significant decrease in the specific activity of the acidic alpha-D-mannosidase (pH 4.2) compared with normal controls. Additional studies revealed a small, but significant, decrease in the rate of degradation of 125I-labelled beta-D-glucosidase that had been endocytosed into Chediak-Higashi cells.


2020 ◽  
Author(s):  
Bruno Oliveira Ferreira de Souza ◽  
Éve‐Marie Frigon ◽  
Robert Tremblay‐Laliberté ◽  
Christian Casanova ◽  
Denis Boire

Author(s):  
Dean A. Handley ◽  
Cynthia M. Arbeeny ◽  
Larry D. Witte

Low density lipoproteins (LDL) are the major cholesterol carrying particles in the blood. Using cultured cells, it has been shown that LDL particles interact with specific surface receptors and are internalized via a coated pit-coated vesicle pathway for lysosomal catabolism. This (Pathway has been visualized using LDL labeled to ferritin or colloidal gold. It is now recognized that certain lysomotropic agents, such as chloroquine, inhibit lysosomal enzymes that degrade protein and cholesterol esters. By interrupting cholesterol ester hydrolysis, chloroquine treatment results in lysosomal accumulation of cholesterol esters from internalized LDL. Using LDL conjugated to colloidal gold, we have examined the ultrastructural effects of chloroquine on lipoprotein uptake by normal cultured fibroblasts.


2001 ◽  
Vol 268 (6) ◽  
pp. 1739-1748
Author(s):  
Aitor Hierro ◽  
Jesus M. Arizmendi ◽  
Javier De Las Rivas ◽  
M. Angeles Urbaneja ◽  
Adelina Prado ◽  
...  

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