کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
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1939783 | 1050767 | 2006 | 6 صفحه PDF | دانلود رایگان |

We herein isolated a peroxisome-deficient Chinese hamster ovary mutant, ZPEG252, import-defective of peroxisome targeting signal 1 (PTS1)- and PTS2-proteins at 37 °C. The impaired protein import was restored at 30 °C, indicating a temperature-sensitive phenotype, similar to that of cells derived from patients with milder peroxisome biogenesis disorders such as infantile Refsum disease. PEX1 expression complemented the mutant phenotype of ZPEG252. Reverse transcription-PCR analysis indicated one point mutation at nucleotide residue 1817 changing a codon (GGG) for Gly606 to a codon (GAG) for Glu606 in the sequence for the Walker A1 motif of the AAA cassettes. This novel mutant Pex1pG606E was severely affected in binding to Pex6p at 37 °C, but not at 30 °C. Pex1pG606E was localized to peroxisomes at 30 °C, whilst it was discernible in a cytosolic staining pattern at 37 °C. Together, our findings demonstrate that Walker A1 motif of Pex1p is essential for Pex1p–Pex6p interaction and Pex1p targeting to peroxisomes.
Journal: Biochemical and Biophysical Research Communications - Volume 345, Issue 4, 14 July 2006, Pages 1434–1439