A soluble form of prion protein in human cerebrospinal fluid: implications for prion-related encephalopathies.
The cellular prion protein (PrPc) is a 33-35 kDa sialoglycoprotein anchored to the external surface of neural and non-neural cells by a glycosyl phosphatidylinositol moiety. In addition, a secretory form of PrPc has been found in cell-free translation systems and in cell cultures. On this basis, we investigated human cerebrospinal fluid for the presence of soluble PrP and identified a protein whose molecular weight, antigenic determinants, N-terminal amino acid sequence and sensitivity to protease digestion corresponded to those of PrPc. In prion-related encephalopathies of humans and animals, the secretory form of PrPc might be converted into the abnormal isoform PrPSc and play a role in the dissemination of the disease process and amyloid formation.
Tagliavini
F
F
Istituto Neurologico Carlo Besta, Milano, Italy.
Prelli
F
F
Porro
M
M
Salmona
M
M
Bugiani
O
O
Frangione
B
B
D000293:Adolescent; D000328:Adult; D000595:Amino Acid Sequence; D000906:Antibodies; D015415:Biomarkers / Q000134:cerebrospinal fluid; D001927:Brain Diseases / Q000134:cerebrospinal fluid / Q000175:diagnosis*; D002675:Child, Preschool; D000939:Epitopes / Q000032:analysis; D006801:Humans; D008562:Membrane Glycoproteins / Q000134:cerebrospinal fluid; D008969:Molecular Sequence Data; D008970:Molecular Weight; D010455:Peptides / Q000138:chemical synthesis / Q000276:immunology; D018620:PrPSc Proteins; D011328:Prions / Q000134:cerebrospinal fluid* / Q000302:isolation & purification; D012995:Solubility
D000906:Antibodies; D015415:Biomarkers; D000939:Epitopes; D008562:Membrane Glycoproteins; D010455:Peptides; D018620:PrPSc Proteins; D011328:Prions