TY - JOUR
T1 - The number of octapeptide repeat affects the expression and conversion of prion protein
AU - Hiraga, Chihiro
AU - Kobayashi, Atsushi
AU - Kitamoto, Tetsuyuki
N1 - Funding Information:
We thank B. Bell for critical review of the manuscript and K. Abe for technical assistance. This study was supported by the Program for Promotion of Fundamental Studies in Health Sciences of National Institute of Biomedical Innovation (T.K.), a Grant from the Ministry of Health, Labor and Welfare (A.K. and T.K.), and a Grant-in-Aid for Scientific Research from the Ministry of Education, Culture, Sports, Science and Technology (A.K. and T.K).
PY - 2009/5/15
Y1 - 2009/5/15
N2 - 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.
AB - 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.
KW - Conversion
KW - Creutzfeldt-Jakob disease
KW - Insertional mutation
KW - Octapeptide repeat
KW - Prion protein
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U2 - 10.1016/j.bbrc.2009.03.093
DO - 10.1016/j.bbrc.2009.03.093
M3 - Article
C2 - 19318088
AN - SCOPUS:64549145015
VL - 382
SP - 715
EP - 719
JO - Biochemical and Biophysical Research Communications
JF - Biochemical and Biophysical Research Communications
SN - 0006-291X
IS - 4
ER -
