alpha-Hemoglobin stabilizing protein is not a suitable marker for a screening test for variant Creutzfeldt-Jakob disease.
BACKGROUND
A test is needed to identify blood donors who are in the preclinical phase of variant Creutzfeldt-Jakob disease (CJD). alpha-Hemoglobin stabilizing protein (AHSP; syn. ERAF, EDRF) transcript levels are reduced in the blood of mice incubating transmissible spongiform encephalopathy.


METHODS
Quantitative real-time polymerase chain reaction and enzyme-linked immunosorbent assay were used to measure AHSP transcript and protein levels in normal blood donors, patients with CJD, and patients with other neuronal and hematologic diseases. Temporal AHSP expression was measured in sheep incubating bovine spongiform encephalopathy (BSE).


RESULTS
Quantitation of AHSP in peripheral blood from normal blood donors revealed that protein levels, but not transcript levels, are influenced by sex with higher levels found in males, suggesting posttranslational regulation involving the product of an X-linked gene. When AHSP mRNA and protein levels were quantitated in peripheral blood from patients with variant and sporadic CJD, no consistent differences from normal were found. Serial quantitation of AHSP in individual BSE-infected sheep did not reveal any disease-related changes.


CONCLUSIONS
We conclude that quantitation of AHSP is not likely to be useful for detection of preclinical prion disease in man.
Appleford
Nigel E J
NE
Bristol Institute for Transfusion Sciences, National Blood Service, Bristol, UK.
Wilson
Kim
K
Houston
Fiona
F
Bruce
Lesley J
LJ
Morrison
Alex
A
Bishop
Matthew
M
Chalmers
Kevin
K
Miele
Gino
G
Massey
Edwin
E
Prowse
Chris
C
Manson
Jean
J
Will
Robert G
RG
Clinton
Michael
M
MacGregor
Ian
I
Anstee
David J
DJ
D018798:Anemia, Iron-Deficiency / Q000097:blood / Q000175:diagnosis; D000818:Animals; D015415:Biomarkers / Q000097:blood*; D001782:Blood Donors; D001798:Blood Proteins / Q000235:genetics; D002417:Cattle; D007562:Creutzfeldt-Jakob Syndrome / Q000097:blood* / Q000175:diagnosis*; D016643:Encephalopathy, Bovine Spongiform / Q000097:blood / Q000175:diagnosis; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006432:Hemochromatosis / Q000097:blood / Q000175:diagnosis; D006801:Humans; D015477:Leukemia, Myelomonocytic, Chronic / Q000097:blood / Q000175:diagnosis; D008297:Male; D008403:Mass Screening / Q000379:methods*; D018832:Molecular Chaperones / Q000097:blood* / Q000235:genetics; D009436:Neural Tube Defects / Q000097:blood / Q000175:diagnosis; D020641:Polymorphism, Single Nucleotide; D011164:Porphyrias / Q000097:blood / Q000175:diagnosis; D011328:Prions / Q000097:blood / Q000235:genetics; D011401:Promoter Regions, Genetic / Q000235:genetics; D012333:RNA, Messenger / Q000097:blood; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012756:Sheep
C468768:AHSP protein, human; D015415:Biomarkers; D001798:Blood Proteins; D018832:Molecular Chaperones; D011328:Prions; D012333:RNA, Messenger