Newly identified Chinese hamster ovary cell mutants defective in peroxisome assembly represent complementation group A of human peroxisome biogenesisdisorders and one novel group in mammals
K. Ghaedi et al., Newly identified Chinese hamster ovary cell mutants defective in peroxisome assembly represent complementation group A of human peroxisome biogenesisdisorders and one novel group in mammals, EXP CELL RE, 248(2), 1999, pp. 482-488
We isolated peroxisome biogenesis-defective mutants from rat PEX2-transform
ed Chinese hamster ovary (CHO) cells, using the 9-(1'-pyrene)nonanol/ultrav
iolet method. A total of 18 mutant cell clones showing cytosolic localizati
on of catalase were isolated. By complementation group (CG;) analysis by me
ans of PEX cDNA transfection and cell fusion, cell mutants, ZP124 and ZP126
, were found to belong to two novel CGs of CHO mutants. Mutants, ZP135 and
ZP167, mere also classified to the same CG as ZP124. Further cell fusion an
alysis using 12 CGs fibroblasts from patients with peroxisome deficiency di
sorders such as Zellweger syndrome revealed that ZP124 belonged to human CG
-A, the same group as CC-WI in the United States. ZP126 could not be classi
fied to any of human and CHO CGs. These mutants also showed typical peroxis
ome assembly-defective phenotypes such as severe loss of catalase latency a
nd impaired biogenesis of peroxisomal enzymes. Collectively, ZP124 represen
ts CG-A, and ZP126 is in a newly identified CG distinct from the 14 mammali
an CGs previously characterized, (C) 1999 Academic Press.