Alpha haemoglobin-stabilising protein concentration in the red blood cells of patients with sickle cell anaemia with and without hydroxycarbamide treatment.
Adult
Anemia, Sickle Cell
/ diagnosis
Biomarkers
Blood Proteins
/ metabolism
Case-Control Studies
Enzyme-Linked Immunosorbent Assay
Erythrocyte Indices
Erythrocytes
/ metabolism
Female
Hemoglobins
/ metabolism
Humans
Hydroxyurea
/ administration & dosage
Male
Middle Aged
Molecular Chaperones
/ metabolism
Peptide Fragments
/ metabolism
Prognosis
ELISA
alpha haemoglobin-stabilising protein
alpha-haemoglobin pool
hydroxycarbamide
sickle cell anaemia
Journal
British journal of haematology
ISSN: 1365-2141
Titre abrégé: Br J Haematol
Pays: England
ID NLM: 0372544
Informations de publication
Date de publication:
01 2022
01 2022
Historique:
revised:
02
07
2021
received:
04
05
2021
accepted:
12
07
2021
pubmed:
12
8
2021
medline:
15
2
2022
entrez:
11
8
2021
Statut:
ppublish
Résumé
Alpha haemoglobin-stabilising protein (AHSP) is a key chaperone synthesised in red blood cell (RBC) precursors. Many studies have reported AHSP as a potential biomarker of various diseases. AHSP gene expression has been studied in detail, but little is known about AHSP protein levels in RBCs. We investigated the AHSP concentration of RBC lysates from control subjects (n = 10) and patients with sickle cell anaemia (SCA) with (n = 10) and without (n = 12) hydroxycarbamide (HC) treatment, to evaluate the clinical relevance of AHSP in SCA. We developed a sandwich enzyme-linked immunosorbent assay method, with which we were able, for the first time, to determine the mean AHSP concentration in control RBC lysates (0·82 µg/ml). The AHSP concentration (2·23 µg/ml) was significantly higher in untreated patients with the SS genotype than in controls. The AHSP concentration decreased significantly on HC treatment (1·50 µg/ml) but remained significantly higher than that in controls. A strong positive correlation was observed between the AHSP concentration and the α-haemoglobin pool with the three groups of subjects pooled into a single group. Our present findings indicate that AHSP concentration can be considered a candidate biomarker for monitoring HC responses in patients with SCA and suggest a role for AHSP in various RBC diseases.
Substances chimiques
AHSP protein, human
0
Biomarkers
0
Blood Proteins
0
Hemoglobins
0
Molecular Chaperones
0
Peptide Fragments
0
alpha(A) globin
137597-31-0
Hydroxyurea
X6Q56QN5QC
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
183-192Informations de copyright
© 2021 British Society for Haematology and John Wiley & Sons Ltd.
Références
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