Defects in PEX1, a peroxisome-associated ATPase that interacts with PEX5 and PEX6, disrupt peroxisomal matrix protein import and are the most common cause of the peroxisome biogenesis disorders.

被引：0

作者：

Reuber, BE

GermainLee, E

Morrell, JC

Geisbrecht, B

Collins, C

Ameritunga, R

Moser, HW

Valle, D

Gould, SJ

机构：

[1] JOHNS HOPKINS UNIV,SCH MED,DEPT BIOL CHEM,BALTIMORE,MD 21205

[2] JOHNS HOPKINS UNIV,SCH MED,HOWARD HUGHES MED INST,DEPT PEDIAT,BALTIMORE,MD 21205

[3] JOHNS HOPKINS UNIV,SCH MED,PREDOCTORAL TRAINING PROGRAM HUMAN GENET,BALTIMORE,MD 21205

[4] JOHNS HOPKINS UNIV,SCH MED,KENNEDY KRIEGER INST,BALTIMORE,MD 21205

[5] JOHNS HOPKINS UNIV,SCH MED,DEPT NEUROL,BALTIMORE,MD 21205

[6] JOHNS HOPKINS UNIV,SCH MED,DEPT MOL BIOL & GENET,BALTIMORE,MD 21205

来源：

MOLECULAR BIOLOGY OF THE CELL | 1997年 / 8卷

关键词：

D O I：

暂无

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

引用

页码：668 / 668

页数：1

共 27 条

[21] A common PEX1 frameshift mutation in patients with disorders of peroxisome biogenesis correlates with the severe Zellweger syndrome phenotype
Maxwell, MA
Nelson, PV
Chin, SJ
Paton, BC
Carey, WF
Crane, DI
HUMAN GENETICS, 1999, 105 (1-2) : 38 - 44
[22] pex5 Mutants That Differentially Disrupt PTS1 and PTS2 Peroxisomal Matrix Protein Import in Arabidopsis
Khan, Bibi Rafeiza
Zolman, Bethany K.
PLANT PHYSIOLOGY, 2010, 154 (04) : 1602 - 1615
[23] The di-aromatic pentapeptide repeats of the human peroxisome import receptor PEX5 are separate high affinity binding sites for the peroxisomal membrane protein PEX14
Saidowsky, J
Dodt, G
Kirchberg, K
Wegner, A
Nastainczyk, W
Kunau, WH
Schliebs, W
JOURNAL OF BIOLOGICAL CHEMISTRY, 2001, 276 (37) : 34524 - 34529
[24] Peroxisomal matrix protein import -: Suppression of protein import defects in Hansenula polymorpha pex mutants by overproduction of the PTS1 receptor pex5p
Kiel, JAKW
Veenhuis, M
CELL BIOCHEMISTRY AND BIOPHYSICS, 2000, 32 (1-3) : 9 - 19
[25] Peroxisomal matrix protein importSuppression of protein import defects in Hansenula polymorpha pex Mutants by Overproduction of the PTS1 Receptor Pex5p
Jan A. K. W. Kiel
Marten Veenhuis
Cell Biochemistry and Biophysics, 2000, 32 : 9 - 19
[26] BIOL 25-Peroxisome biogenesis disorders: Peroxisomal targeting signal-1 binding to Pex5-C
Ghosh, Debdip
Maynard, Ernest L.
Berg, Jeremy M.
ABSTRACTS OF PAPERS OF THE AMERICAN CHEMICAL SOCIETY, 2008, 235
[27] Mutations in the peroxin Pex26p responsible for peroxisome biogenesis disorders of complementation group 8 impair its stability, peroxisomal localization, and interaction with the Pex1p•Pex6p complex
Furuki, S
Tamura, S
Matsumoto, N
Miyata, N
Moser, A
Moser, HW
Fujiki, Y
JOURNAL OF BIOLOGICAL CHEMISTRY, 2006, 281 (03) : 1317 - 1323

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