alpha-Hemoglobin stabilizing protein is not a suitable marker for a screening test for variant Creutzfeldt-Jakob disease

Nigel E. J. Appleford, Kim Wilson, Fiona Houston, Lesley J. Bruce, Alex Morrison, Matthew Bishop, Kevin Chalmers, Gino Miele, Edwin Massey, Chris Prowse, Jean Manson, Robert G. Will, Michael Clinton, Ian MacGregor, David J. Anstee

Research output: Contribution to journalArticlepeer-review


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.

STUDY DESIGN AND 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.

CONCLUSION: We conclude that quantitation of AHSP is not likely to be useful for detection of preclinical prion disease in man.

Original languageEnglish
Pages (from-to)1616-1626
Number of pages11
Issue number8
Publication statusPublished - Aug 2008

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