Alpha-Hemoglobin-Stabilizing Protein: An Erythroid Molecular Chaperone
Alpha-hemoglobin-stabilizing protein (AHSP) is an erythroid-specific protein that acts as a molecular chaperone for the free α chains of hemoglobin. Evidence strongly suggests that AHSP participates in hemoglobin synthesis and may act to neutralize the cytotoxic effects of excess free alpha-globin s...
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| Format: | Article |
| Language: | English |
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Wiley
2011-01-01
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| Series: | Biochemistry Research International |
| Online Access: | http://dx.doi.org/10.1155/2011/373859 |
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| author | Maria Emília Favero Fernando Ferreira Costa |
| author_facet | Maria Emília Favero Fernando Ferreira Costa |
| author_sort | Maria Emília Favero |
| collection | DOAJ |
| description | Alpha-hemoglobin-stabilizing protein (AHSP) is an erythroid-specific protein that acts as a molecular chaperone for the free α chains of hemoglobin. Evidence strongly suggests that AHSP participates in hemoglobin synthesis and may act to neutralize the cytotoxic effects of excess free alpha-globin subunits that accumulate both in normal and beta-thalassemic erythroid precursor cells. As such, AHSP seems to be essential for normal erythropoiesis, and impaired upregulation of AHSP may lead to premature erythroid cell death, resulting in ineffective erythropoiesis. Reduced AHSP mRNA expression has been associated with clinical variability in some cases of β-thalassemia. It has been shown that αHb variants may also impair AHSP-αHb interactions, leading to pathological conditions that resemble α-thalassemia syndromes. The aim of this paper is to summarize current information concerning the structure and function of AHSP, focusing on its role in normal erythropoiesis and its relevance in health and disease. |
| format | Article |
| id | doaj-art-fc303f6bbd8b4e49816ce055df8f8a3a |
| institution | Kabale University |
| issn | 2090-2247 2090-2255 |
| language | English |
| publishDate | 2011-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Biochemistry Research International |
| spelling | doaj-art-fc303f6bbd8b4e49816ce055df8f8a3a2025-02-03T06:01:39ZengWileyBiochemistry Research International2090-22472090-22552011-01-01201110.1155/2011/373859373859Alpha-Hemoglobin-Stabilizing Protein: An Erythroid Molecular ChaperoneMaria Emília Favero0Fernando Ferreira Costa1Department of Pathology, Clinical Analysis and Toxicology, University Hospital, State University of Londrina (UEL), Avenida Robert Koch, 60, Vila Operária, 86038-350 Londrina, PR, BrazilHematology and Hemotherapy Center, State University of Campinas (UNICAMP), Rua Carlos Chagas, 480, Cidade Universitária, Barão Geraldo, 13083-970, Campinas SP, BrazilAlpha-hemoglobin-stabilizing protein (AHSP) is an erythroid-specific protein that acts as a molecular chaperone for the free α chains of hemoglobin. Evidence strongly suggests that AHSP participates in hemoglobin synthesis and may act to neutralize the cytotoxic effects of excess free alpha-globin subunits that accumulate both in normal and beta-thalassemic erythroid precursor cells. As such, AHSP seems to be essential for normal erythropoiesis, and impaired upregulation of AHSP may lead to premature erythroid cell death, resulting in ineffective erythropoiesis. Reduced AHSP mRNA expression has been associated with clinical variability in some cases of β-thalassemia. It has been shown that αHb variants may also impair AHSP-αHb interactions, leading to pathological conditions that resemble α-thalassemia syndromes. The aim of this paper is to summarize current information concerning the structure and function of AHSP, focusing on its role in normal erythropoiesis and its relevance in health and disease.http://dx.doi.org/10.1155/2011/373859 |
| spellingShingle | Maria Emília Favero Fernando Ferreira Costa Alpha-Hemoglobin-Stabilizing Protein: An Erythroid Molecular Chaperone Biochemistry Research International |
| title | Alpha-Hemoglobin-Stabilizing Protein: An Erythroid Molecular Chaperone |
| title_full | Alpha-Hemoglobin-Stabilizing Protein: An Erythroid Molecular Chaperone |
| title_fullStr | Alpha-Hemoglobin-Stabilizing Protein: An Erythroid Molecular Chaperone |
| title_full_unstemmed | Alpha-Hemoglobin-Stabilizing Protein: An Erythroid Molecular Chaperone |
| title_short | Alpha-Hemoglobin-Stabilizing Protein: An Erythroid Molecular Chaperone |
| title_sort | alpha hemoglobin stabilizing protein an erythroid molecular chaperone |
| url | http://dx.doi.org/10.1155/2011/373859 |
| work_keys_str_mv | AT mariaemiliafavero alphahemoglobinstabilizingproteinanerythroidmolecularchaperone AT fernandoferreiracosta alphahemoglobinstabilizingproteinanerythroidmolecularchaperone |