Kinetics of the assembly of peroxisomes after fusion of complementary cell lines from patients with the cerebro-hepato-renal (Zellweger) syndrome and related disorders.
We have recently identified four complementation groups in fibroblasts from patients deficient in peroxisomes. Here we describe a kinetic analysis of the complementation process. The kinetics of peroxisome assembly was assessed in heterokaryons of complementary cell lines by measuring the rate of incorporation of catalase, initially present in the cytosol, into particles. In two combinations of cell lines assembly was rapid and insensitive to cycloheximide. Thus the components required for peroxisome assembly must have been present in the parental cell lines, at least one of which presumably contained peroxisomal ghosts. In three other combinations of cell lines assembly of peroxisomes was slow and sensitive to cycloheximide.
Brul
S
S
Laboratory of Biochemistry, University of Amsterdam, The Netherlands.
Wiemer
E A
EA
Westerveld
A
A
Strijland
A
A
Wanders
R J
RJ
Schram
A W
AW
Heymans
H S
HS
Schutgens
R B
RB
Van den Bosch
H
H
Tager
J M
JM
D001927:Brain Diseases; D002374:Catalase / Q000378:metabolism; D002459:Cell Fusion; D002460:Cell Line; D003513:Cycloheximide / Q000494:pharmacology; D005347:Fibroblasts; D006801:Humans; D007674:Kidney Diseases; D008107:Liver Diseases; D008830:Microbodies / Q000187:drug effects / Q000201:enzymology / Q000648:ultrastructure*; D013577:Syndrome
D003513:Cycloheximide; D002374:Catalase