Total prion protein levels in the cerebrospinal fluid are reduced in patients with various neurological disorders.
We performed a study on levels of the total prion protein (PrP) in humans affected by different neurological diseases and assessed the influence of several factors such as age, gender, and disease severity on the cerebrospinal fluid PrP levels. PrP-ELISA technique was used to analyze cerebrospinal fluid (CSF) samples. 293 CSF samples of patients with Creutzfeldt-Jakob-disease (CJD), Alzheimer's disease, dementia with Lewy-bodies, Parkinson's disease, multiple sclerosis, cerebral ischemia, generalized epileptic seizures, and meningitis and encephalitis in comparison to controls were analyzed. We found a significant reduction of CSF PrP levels in patients suffering from all neurodegenerative disorders analyzed. This group exhibited mean PrP values of 164 ng/ml while non-neurodegenerative disorder patients and healthy controls showed PrP levels of 208 ng/ml and 226 ng/ml, respectively. CSF levels correlated with disease severity in CJD, Alzheimer's disease, and dementia with Lewy-bodies. The finding of decreased PrP levels in the CSF of patients not only with CJD but also in other neurodegenerative disorders is intriguing. Age-, gender-, and genetic-specific factors might be involved in the PrP c regulation.
Meyne
Felix
F
National TSE Reference Center at Department of Neurology, Georg-August University, Göttingen, Germany.
Gloeckner
Sara Friederike
SF
Ciesielczyk
Barbara
B
Heinemann
Uta
U
Krasnianski
Anna
A
Meissner
Bettina
B
Zerr
Inga
I
D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D000544:Alzheimer Disease / Q000134:cerebrospinal fluid; D002545:Brain Ischemia / Q000134:cerebrospinal fluid; D007562:Creutzfeldt-Jakob Syndrome / Q000134:cerebrospinal fluid; D004660:Encephalitis / Q000134:cerebrospinal fluid; D004797:Enzyme-Linked Immunosorbent Assay; D004829:Epilepsy, Generalized / Q000134:cerebrospinal fluid; D005260:Female; D006801:Humans; D020961:Lewy Body Disease / Q000134:cerebrospinal fluid; D008297:Male; D008581:Meningitis / Q000134:cerebrospinal fluid; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000134:cerebrospinal fluid; D009422:Nervous System Diseases / Q000134:cerebrospinal fluid* / Q000503:physiopathology; D010300:Parkinson Disease / Q000134:cerebrospinal fluid; D011328:Prions / Q000134:cerebrospinal fluid*; D012720:Severity of Illness Index; D012737:Sex Factors
D011328:Prions