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Table 1 Markers in patients with prion diseases

From: Genomic and post-genomic analyses of human prion diseases

   Relative levels in human prion disease compared to controls   
Marker Cellular function CSF Plasma Reference Notes
α1-ACT Serine proteinase inhibitor ↑ sCJD ↔ sCJD [54] Elevated in urine of sCJD and in plasma and CSF of AD
   ↑ sCJD NT [63]  
α-Tocopherol Major lipophilic antioxidant ↓ sCJD ↔ sCJD [64] -
Apolipoprotein Components of high density lipoprotein in plasma. Participate in PrP clustering and sequestration ↔ sCJD NT [65] Over-expression of Apo-J in CJD brains and in urine of BSE orally-infected cattle
   ↑ vCJD compared to sCJD NT [66]  
   ↑ sCJD (A1 and A4) NT [63]  
   ↓ sCJD (Apo-J, Clusterin) NT [63]  
Ascorbate Major hydrophilic antioxidant ↓ sCJD ↓ sCJD [64] -
C-reactive protein Markers for inflammation or tissue injury NT ↔ sCJD [67] -
Cystatin C A cysteine proteinase inhibitor, mostly synthesized in the CSF; it is also localized in glial cells and neurons ↑ CJD (not specified) NT [68] Gene is upregulated in the brain of sCJD; reported normal in AD patients
   ↑ sCJD NT [63]  
   ↑ sCJD, ↔ vCJD NT [54]  
F2-isoprostanes Markers of lipid peroxidation and oxidative stress in vivo ↑ sCJD, ↑ gCJD NT [48] -
   ↔ vCJD NT [49]  
Gelsolin Regulator of actin filament assembly ↓ sCJD NT [63] No difference between CJD and AD
   ↓ sCJD NT [46]  
H-FABP Belonging to a family of small, highly conserved, cytosolic proteins involved in fatty acid transport and metabolism ↑ sCJD, ↑ vCJD ↑ sCJD, ↑ vCJD [55] CSF of CJD taken post-mortem while in controls taken from living individuals; plasma levels do not differ between CJD and AD
   ↑ sCJD ↑ sCJD [56]  
   ↑ sCJD NT [69]  
Hp2-α haptoglobin Binds hemoglobin for physiological degradation ↑ sCJD NT [63] -
Interleukin 1β Pro-inflammatory cytokine, involved in immune response ↑ sCJD, ↑ vCJD NT [70] -
Interleukin 4 and 10 Anti-inflammatory cytokine ↑ sCJD NT [71] Not altered in the brain of sCJD
Interleukin 6 Markers for inflammation or tissue injury NT ↔ sCJD [67] -
Interleukin 8 Chemokine with immunoreactive s propertie ↑ sCJD NT [71] CJD Increased in AD, but at a lower level than
Lactic acid End-product of anaerobic glycolysis ↑ sCJD NT [72] Produced by LDH action on pyruvate
LDH Catalyzes the interconversion between pyrvate and lactic acid ↑ sCJD NT [46, 47] -
MDA Marker of oxidative stress ↔ sCJD ↔ sCJD (serum) [73] Increased MDA has been reported in scrapie-infected mice
PGE2 Major arachidonic acid metabolite of the cyclooxygenase pathway ↑ sCJD, ↑ gCJD NT [48] Levels of PGE2 correlate with disease duration in sCJD
   ↑ vCJD   [49]  
TGF-β2 Anti-inflammatory cytokine ↓ sCJD NT [71] Increased immunoreactivity in neurons of the neurocortex in 20 patients with human prion diseases
TNF-α Proinflammatory cytokine ↑ sCJD, ↑ vCJD NT [70] Gene is upregulated in the brain of sCJD patients
   Not detectable, sCJD NT [71]  
Transferrin Iron carrier protein in the blood ↑ sCJD NT [63] Upregulated in sera of patients with AD
   ↑ sCJD NT [46]  
Ubiquitin Involved in ATP-dependent selective degradation of cellular proteins, maintenance of chromatin structure, regulation of gene expression, stress response, and ribosome biogenesis. ↑ sCJD NT [63] Elevated levels in CSF of AD patients
Uric acid Non-enzymatic antioxidant in the brain ↔ sCJD, ↓ vCJD NT [50] Decreased level in the CSF in BSE-infected cattle
  1. Arrows indicate: up, increased; down, decreased; horizontal, equivalent. A1, apolipoprotein 1; A4, apolipoprotein 4; α1-ACT, antichymotrypsin; AD, Alzheimer's disease; Apo-J, apolipoprotein J or clusterin; ATP, adenosine triphosphate; BSE, bovine spongiform encephalopathy; gCJD, genetic Creutzfeldt-Jakob disease; sCJD, sporadic Creutzfeldt-Jakob disease; vCJD, variant Creutzfeldt-Jakob disease; CSF, cerebrospinal fluid; H-FABP, heart-2, transforming growth fatty acid binding protein; LDH, lactate dehydrogenase; MDA, malondialdehyde; NT, not tested; PGE2, prostaglandin E2; TGF-β factor-β2; TNF-α, tumor necrosis factor-α.