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Prion. 2013 Sep-Oct;7(5):383-93. doi: 10.4161/pri.26416. Epub 2013 Sep 18.

PrP mRNA and protein expression in brain and PrP(c) in CSF in Creutzfeldt-Jakob disease MM1 and VV2.

Author information

1
Institute of Neuropathology; IDIBELL-University Hospital Bellvitge; University of Barcelona; Hospitalet de Llobregat; Barcelona, Spain; CIBERNED (Network Center for Biomedical Research of Neurodegenerative Diseases); Institute Carlos III; Ministry of Health; Madrid, Spain; Department of Neurology; Clinical Dementia Center and DZNE; University Medical School; Georg-August University; Göttingen, Germany.
2
Institute of Neuropathology; IDIBELL-University Hospital Bellvitge; University of Barcelona; Hospitalet de Llobregat; Barcelona, Spain; CIBERNED (Network Center for Biomedical Research of Neurodegenerative Diseases); Institute Carlos III; Ministry of Health; Madrid, Spain.
3
Department of Neurology; Clinical Dementia Center and DZNE; University Medical School; Georg-August University; Göttingen, Germany.
4
CJD-Unit and Alzheimer disease and Other Cognitive Disorders Unit; Department of Neurology; Hospital Clínic; Barcelona, Spain.
5
CJD-Unit and Alzheimer disease and Other Cognitive Disorders Unit; Department of Neurology; Hospital Clínic; Barcelona, Spain; Department of Immunology; Hospital Clinic; Barcelona, Spain.
6
General Subdirectorate of Surveillance and Response to Emergencies in Public Health; Department of Public Health in Catalonia; Barcelona, Spain.
7
CIBERNED (Network Center for Biomedical Research of Neurodegenerative Diseases); Institute Carlos III; Ministry of Health; Madrid, Spain; Molecular and Cellular Neurobiotechnology; Catalonian Institute for Bioengineering (IBEC); Parc Científic de Barcelona; Barcelona, Spain; Department of Cell Biology; University of Barcelona; Barcelona, Spain.
8
Neurological Tissue Bank of the Biobanc-Hospital; Clínic-Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS); Barcelona, Spain.

Abstract

Creutzfeldt-Jakob disease (CJD) is a heterogenic neurodegenerative disorder associated with abnormal post-translational processing of cellular prion protein (PrP(c)). CJD displays distinctive clinical and pathological features which correlate with the genotype at the codon 129 (methionine or valine: M or V respectively) in the prion protein gene and with size of the protease-resistant core of the abnormal prion protein PrP(sc) (type 1: 20/21 kDa and type 2: 19 kDa). MM1 and VV2 are the most common sporadic CJD (sCJD) subtypes. PrP mRNA expression levels in the frontal cortex and cerebellum are reduced in sCJD in a form subtype-dependent. Total PrP protein levels and PrP(sc) levels in the frontal cortex and cerebellum accumulate differentially in sCJD MM1 and sCJD VV2 with no relation between PrP(sc) deposition and spongiform degeneration and neuron loss, but with microgliosis, and IL6 and TNF-α response. In the CSF, reduced PrP(c), the only form present in this compartment, occurs in sCJD MM1 and VV2. PrP mRNA expression is also reduced in the frontal cortex in advanced stages of Alzheimer disease, Lewy body disease, progressive supranuclear palsy, and frontotemporal lobe degeneration, but PrP(c) levels in brain varies from one disease to another. Reduced PrP(c) levels in CSF correlate with PrP mRNA expression in brain, which in turn reflects severity of degeneration in sCJD.

KEYWORDS:

Creutzfeldt-Jakob disease; brain; cerebrospinal fluid; mRNA; neurodegenerative diseases; prion protein

PMID:
24047819
PMCID:
PMC4134343
DOI:
10.4161/pri.26416
[Indexed for MEDLINE]
Free PMC Article

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