The genetics of peroxisome biogenesis

被引:87
作者
Sacksteder, KA [1 ]
Gould, SJ
机构
[1] Johns Hopkins Univ, Sch Med, Dept Biol Chem, Baltimore, MD 21205 USA
[2] Johns Hopkins Univ, Sch Med, Dept Cell Biol & Anat, Baltimore, MD 21205 USA
关键词
organelle assembly; peroxins; pex genes; protein import; Zellweger syndrome;
D O I
10.1146/annurev.genet.34.1.623
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
The segregation of metabolic functions within discrete organelles is a hallmark of eukaryotic cells. These compartments allow for the concentration of related metabolic functions, the separation of competing metabolic functions, and the formation of unique chemical microenvironments. However, such organization is not spontaneous and requires an array of genes that are dedicated to the assembly and maintenance of these structures. In this review we focus on the genetics of peroxisome biogenesis and on how defects in this process cause human disease.
引用
收藏
页码:623 / 652
页数:30
相关论文
共 169 条
[1]   cDNA cloning and characterization of a constitutively expressed isoform of the human peroxin Pex11p [J].
Abe, I ;
Fujiki, Y .
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 1998, 252 (02) :529-533
[2]   Clofibrate-inducible, 2X-kDa peroxisomal integral membrane protein is encoded by PEX11 [J].
Abe, I ;
Okumoto, K ;
Tamura, S ;
Fujiki, Y .
FEBS LETTERS, 1998, 431 (03) :468-472
[3]   Pex14p, a peroxisomal membrane protein binding both receptors of the two PTS-dependent import pathways [J].
Albertini, M ;
Rehling, P ;
Erdmann, R ;
Girzalsky, W ;
Kiel, JAKW ;
Veenhuis, M ;
Kunau, WH .
CELL, 1997, 89 (01) :83-92
[4]   C-terminal tripeptide Ser-Asn-Leu (SNL) of human D-aspartate oxidase is a functional peroxisome-targeting signal [J].
Amery, L ;
Brees, C ;
Baes, M ;
Setoyama, C ;
Miura, R ;
Mannaerts, GP ;
Van Veldhoven, PP .
BIOCHEMICAL JOURNAL, 1998, 336 :367-371
[5]   The Hansenula polymorpha PER9 gene encodes a peroxisomal membrane protein essential for peroxisome assembly and integrity [J].
Baerends, RJS ;
Rasmussen, SW ;
Hilbrands, RE ;
vanderHeide, M ;
Faber, KN ;
Reuvekamp, PTW ;
Kiel, JAKW ;
Cregg, JM ;
vanderKlei, IJ ;
Veenhuis, M .
JOURNAL OF BIOLOGICAL CHEMISTRY, 1996, 271 (15) :8887-8894
[6]  
Blatch GL, 1999, BIOESSAYS, V21, P932, DOI 10.1002/(SICI)1521-1878(199911)21:11<932::AID-BIES5>3.3.CO
[7]  
2-E
[8]   RING domains: Master builders of molecular scaffolds? [J].
Borden, KLB .
JOURNAL OF MOLECULAR BIOLOGY, 2000, 295 (05) :1103-1112
[9]   Human PEX7 encodes the peroxisomal PTS2 receptor and is responsible for rhizomelic chondrodysplasia punctata [J].
Braverman, N ;
Steel, G ;
Obie, C ;
Moser, A ;
Moser, H ;
Gould, SJ ;
Valle, D .
NATURE GENETICS, 1997, 15 (04) :369-376
[10]   An isoform of Pex5p, the human PTS1 receptor, is required for the import of PTS2 proteins into peroxisomes [J].
Braverman, N ;
Dodt, G ;
Gould, SJ ;
Valle, D .
HUMAN MOLECULAR GENETICS, 1998, 7 (08) :1195-1205