Animal cell mutants represent two complementation groups of peroxisome-defective Zellweger syndrome.
نویسندگان
چکیده
منابع مشابه
Animal cell mutants represent two complementation groups of peroxisome-defective Zellweger syndrome.
Generalized peroxisome-deficient disorders including cerebro-hepato-renal Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum disease are autosomal recessive diseases, where catalase-containing particles (peroxisomes) are morphologically absent. We previously isolated two Chinese hamster ovary (CHO) cell mutants (Z24 and Z65) that resemble the fibroblasts from patients with ...
متن کاملMutations in PEX10 is the cause of Zellweger peroxisome deficiency syndrome of complementation group B.
Peroxisome biogenesis disorders (PBD), such as Zellweger syndrome, are autosomal recessive diseases caused by a deficiency in peroxisome assembly as well as a malfunction of the peroxisomes, where at least 10 genotypes have been reported. We have isolated a human PEX10 cDNA (HsPEX10) by an expressed sequence tag homology search on a human DNA database using yeast PEX10 from Hansenula polymorpha...
متن کاملTransfer-defective mutants of sex factors in Escherichia coli. I. Defective mutants and complementation analysis.
HE fertility factor, F, controls conjugal fertility of Escherichia coli Kl2 ( LEDERBERG, CAVALLI and LEDERBERG 1952; HAYES 1953). F also determines functions such as immunity to superinfection by a second F factor (MAAS 1963) ; growth inhibition of certain phages such as tau ( 7 ) (HAKURA, OTSUJI and HIROTA 1964) , T7 ( MAKEL;~, MAKEL;~ and SOIKKELI 1964), and T3 ( SCHELL et al. 1963) ; and for...
متن کاملIsolation and Characterization of a New Peroxisome Deficient CHO Mutant Cell Belonging to Complementation Group 12
We searched for novel Chinese hamster ovary (CHO) cell mutants defective in peroxisome biogenesis by an improved method using peroxisome targeting sequence (PTS) of Pex3p (amino acid residues 1–40)-fused enhanced green fluorescent protein (EGFP). From mutagenized TKaEG3(1–40) cells, the wild-type CHO-K1 stably expressing rat Pex2p and of rat Pex3p(1–40)-EGFP, numerous cell colonies resistant to...
متن کاملMetabolic control of peroxisome abundance.
Zellweger syndrome and related disorders represent a group of lethal, genetically heterogeneous diseases. These peroxisome biogenesis disorders (PBDs) are characterized by defective peroxisomal matrix protein import and comprise at least 10 complementation groups. The genes defective in seven of these groups and more than 90% of PBD patients are now known. Here we examine the distribution of pe...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Journal of Clinical Investigation
سال: 1992
ISSN: 0021-9738
DOI: 10.1172/jci116063