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alpha-Hemoglobin stabilizing protein is not a suitable marker for a screening test for variant Creutzfeldt-Jakob
Nigel E J Appleford1, Kim Wilson, Fiona Houston
1Bristol Institute for Transfusion Sciences, National Blood Service, Bristol, UK.
Insights
Quantifying alpha-hemoglobin stabilizing protein (AHSP) in blood is not a reliable method for detecting preclinical prion diseases like variant Creutzfeldt-Jakob disease (vCJD) in humans.
Area of Science:
- Biochemistry
- Neuroscience
- Hematology
Background:
- Variant Creutzfeldt-Jakob disease (vCJD) poses a risk to blood supply safety.
- A diagnostic test for preclinical vCJD in blood donors is needed.
- Alpha-hemoglobin stabilizing protein (AHSP) levels were previously observed to decrease in mice with transmissible spongiform encephalopathy.
Purpose of the Study:
- To investigate the potential of AHSP transcript and protein levels as biomarkers for preclinical prion diseases.
- To assess AHSP levels in human blood donors and patients with neurological and hematological diseases.
- To monitor AHSP expression in sheep experimentally infected with bovine spongiform encephalopathy (BSE).
Main Methods:
- Quantitative real-time polymerase chain reaction (qPCR) to measure AHSP mRNA.
- Enzyme-linked immunosorbent assay (ELISA) to quantify AHSP protein.
- Analysis of blood samples from healthy donors, vCJD patients, and patients with other diseases.
- Longitudinal monitoring of AHSP in BSE-infected sheep.
Main Results:
- AHSP protein levels in healthy donors varied by sex, with higher levels in males, suggesting posttranslational regulation.
- No consistent differences in AHSP mRNA or protein levels were found in patients with variant or sporadic CJD compared to controls.
- AHSP levels in BSE-infected sheep did not show disease-related changes.
Conclusions:
- Quantitation of AHSP in peripheral blood is unlikely to be a useful diagnostic tool for detecting preclinical prion diseases in humans.
- Further research may be needed to identify reliable biomarkers for preclinical vCJD detection.
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.
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