The number of octapeptide repeat affects the expression and conversion of prion protein

Chihiro Hiraga; Atsushi Kobayashi; Tetsuyuki Kitamoto

doi:10.1016/j.bbrc.2009.03.093

The number of octapeptide repeat affects the expression and conversion of prion protein

Chihiro Hiraga, Atsushi Kobayashi, Tetsuyuki Kitamoto

MED - United Centers for Advanced Research and Translational Medicine (ART)

Research output: Contribution to journal › Article

2 Citations (Scopus)

Abstract

The human prion protein (PrP) has five copies of an octapeptide repeat (OR). The mutant PrP with 6-14 OR causes the genetic form of Creutzfeldt-Jakob disease (CJD). To determine the influence of OR on the conversion of PrP, we examined the conversion efficiency of mouse mutant PrP molecules with 1-16 OR in scrapie-infected cells. The expression level of mutant PrP and the glycoform ratio of the abnormal isoform of PrP (PrP^Sc) were affected by the number of OR. The conversion efficiency was almost equivalent among mutant PrP molecules with 5-16 OR, whereas that of mutant PrP with 1-4 OR was decreased. The present study suggests that CJD patients with the longer extra OR, who usually show only a trace of PrP^Sc in the brain, can produce the authentic triplet PrP^Sc if secondary prion infection occurs.

Original language	English
Pages (from-to)	715-719
Number of pages	5
Journal	Biochemical and biophysical research communications
Volume	382
Issue number	4
DOIs	https://doi.org/10.1016/j.bbrc.2009.03.093
Publication status	Published - 2009 May 15

Keywords

Conversion
Creutzfeldt-Jakob disease
Insertional mutation
Octapeptide repeat
Prion protein

ASJC Scopus subject areas

Biophysics
Biochemistry
Molecular Biology
Cell Biology

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Hiraga, C., Kobayashi, A., & Kitamoto, T. (2009). The number of octapeptide repeat affects the expression and conversion of prion protein. Biochemical and biophysical research communications, 382(4), 715-719. https://doi.org/10.1016/j.bbrc.2009.03.093

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abstract = "The human prion protein (PrP) has five copies of an octapeptide repeat (OR). The mutant PrP with 6-14 OR causes the genetic form of Creutzfeldt-Jakob disease (CJD). To determine the influence of OR on the conversion of PrP, we examined the conversion efficiency of mouse mutant PrP molecules with 1-16 OR in scrapie-infected cells. The expression level of mutant PrP and the glycoform ratio of the abnormal isoform of PrP (PrPSc) were affected by the number of OR. The conversion efficiency was almost equivalent among mutant PrP molecules with 5-16 OR, whereas that of mutant PrP with 1-4 OR was decreased. The present study suggests that CJD patients with the longer extra OR, who usually show only a trace of PrPSc in the brain, can produce the authentic triplet PrPSc if secondary prion infection occurs.",

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