Truncated PrP(c) in mammalian brain: interspecies variation and location in membrane rafts.
A key molecular event in prion diseases is the conversion of cellular prion protein (PrP(c)) into an abnormal misfolded conformer (PrP(sc)). The PrP(c) N-terminal domain plays a central role in PrP(c) functions and in prion propagation. Because mammalian PrP(c) is found as a full-length and N-terminally truncated form, we examined the presence and amount of PrP(c) C-terminal fragment in the brain of different species. We found important variations between primates and rodents. In addition, our data show that the PrP(c) fragment is present in detergent-resistant raft domains, a membrane domain of critical importance for PrP(c) functions and its conversion into PrP(sc).
Laffont-Proust
Isabelle
I
INSERM Avenir Team-Human prion diseases, IFR70, Neuropathology, Salpêtrière Hospital, F-75013 Paris, France.
Hässig
Raymonde
R
Haïk
Stéphane
S
Simon
Stéphanie
S
Grassi
Jacques
J
Fonta
Caroline
C
Faucheux
Baptiste A
BA
Moya
Kenneth L
KL
D000818:Animals; D001921:Brain / Q000378:metabolism*; D002462:Cell Membrane / Q000378:metabolism*; D006224:Cricetinae; D003902:Detergents / Q000494:pharmacology; D004591:Electrophoresis, Polyacrylamide Gel; D021962:Membrane Microdomains / Q000378:metabolism*; D010215:Papio; D018621:PrPC Proteins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D018620:PrPSc Proteins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D011323:Primates; D017096:Prion Diseases / Q000209:etiology / Q000378:metabolism*; D012377:Rodentia; D013045:Species Specificity
D003902:Detergents; D018621:PrPC Proteins; D018620:PrPSc Proteins