Peroxisome assembly factor 1: nonsense mutation in a peroxisome-deficient Chinese hamster ovary cell mutant and deletion analysis

Peroxisome assembly factor 1: nonsense mutation in a peroxisome-deficient Chinese hamster ovary cell mutant and deletion analysis
复制标题

过氧化物酶体组装因子1:过氧化物酶体缺陷的中国仓鼠卵巢细胞突变体中的无义突变和缺失分析

DOI:
--
复制
发表时间:
1994
影响因子:
5.3
通讯作者:
Yukio Fujiki
Yukio Fujiki
中科院分区:
生物学2区
文献类型:
--
作者:
T. Tsukamoto;N. Shimozawa;Yukio Fujiki

文献摘要

参考文献

被引文献

相似文献

从中国仓鼠卵巢(CHO)细胞中克隆了一个编码35-kDa过氧化体组装因子-1(PAF-1)的基因,并进行了序列测定。CHO PAF-1由304个氨基酸组成,比大鼠和人PAF-1短1个氨基酸,与大鼠和人PAF-1的核苷酸同源性分别为90%和86%,氨基酸同源性分别为92%和90%。这三个物种的PAF-1在C末端含有一个保守的富含半胱氨酸的序列,这与一个新的富含半胱氨酸的环指基序家族的序列完全相同。从过氧酶体缺陷CHO细胞突变体Z65(T.Tsukamoto,S.Yokota,和Y.Fujiki,J.Cell Biol.110:651-660,1990),含有Trp-114密码子的无义突变,导致提前终止。在Z65突变体和Zellweger患者的成纤维细胞中测试时,PAF-1中来自N端的19个氨基酸或C端的92个残基的截断保持了过氧化体组装恢复活性。相反,N或C末端27个或102个残基的缺失消除了该活性。PAF-1由游离多聚体RNA编码,符合包括膜多肽在内的过氧化体蛋白生物发生的一般规律,表明PAF-1翻译后转运和整合到过氧体的膜上。
A cDNA encoding 35-kDa peroxisome assembly factor 1 (PAF-1), a peroxisomal integral membrane protein, was cloned from Chinese hamster ovary (CHO) cells and sequenced. The CHO PAF-1 comprised 304 amino acids, one residue shorter than rat or human PAF-1, and showed high homology to rat and human PAF-1: 90 and 86% at the nucleotide sequence level and 92 and 90% in amino acid sequence, respectively. PAF-1 from these three species contains a conserved cysteine-rich sequence at the C-terminal region which is exactly the same as that of a novel cysteine-rich RING finger motif family. PAF-1 cDNA from a peroxisome-deficient CHO cell mutant, Z65 (T. Tsukamoto, S. Yokota, and Y. Fujiki, J. Cell Biol. 110:651-660, 1990), contained a nonsense mutation at the codon for Trp-114, resulting in premature termination. Truncation in PAF-1 of either 19 amino acids from the N terminus or 92 residues from the C terminus maintained the peroxisome assembly-restoring activity when tested in both the Z65 mutant and the fibroblasts from a Zellweger patient. In contrast, deletion of 27 or 102 residues from the N or C terminus eliminated the activity. PAF-1 is encoded by free polysomal RNA, consistent with a general rule for biogenesis of peroxisomal proteins, including membrane polypeptides, implying the posttranslational transport and integration of PAF-1 into peroxisomal membrane.
巨噬细胞样细胞系中的突变体在缩醛磷脂生物合成方面存在缺陷,但含有功能性过氧化物酶体。
DOI: --
发表时间: 1992
期刊: The Journal of biological chemistry
影响因子: --
作者:
Zoeller,RA;Rangaswamy,S;Herscovitz,H;Rizzo,WB;Hajra,AK;Das,AK;Moser,HW;Moser,A;Lazarow,PB;Santos,MJ
通讯作者: Santos,MJ
中国仓鼠卵巢细胞突变体过氧化物酶体生物合成有缺陷。
DOI: --
发表时间: 1989
期刊: The Journal of biological chemistry
影响因子: --
作者:
Zoeller,RA;Allen,LA;Santos,MJ;Lazarow,PB;Hashimoto,T;Tartakoff,AM;Raetz,CR
通讯作者: Raetz,CR