alpha-Globins

Members of the alpha-globin family. In humans, they are encoded in a gene cluster on CHROMOSOME 16. They include zeta-globin and alpha-globin. There are also pseudogenes of zeta (theta-zeta) and alpha (theta-alpha) in the cluster. Adult HEMOGLOBIN is comprised of 2 alpha-globin chains and 2 beta-globin chains.

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Networked: 481 relevant articles (6 outcomes, 48 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Fucharoen, Suthat: 11 articles (02/2012 - 06/2005)
2.	Harteveld, Cornelis L: 11 articles (08/2010 - 08/2002)
3.	Giordano, Piero C: 10 articles (08/2010 - 08/2002)
4.	Higgs, Douglas R: 9 articles (01/2010 - 06/2003)
5.	Winichagoon, Pranee: 8 articles (05/2010 - 06/2005)
6.	Viprakasit, Vip: 7 articles (03/2010 - 02/2004)
7.	Svasti, Saovaros: 6 articles (02/2012 - 05/2008)
8.	Galanello, Renzo: 5 articles (01/2014 - 09/2009)
9.	Weiss, Mitchell J: 5 articles (10/2010 - 10/2002)
10.	Kanavakis, Emmanuel: 5 articles (09/2009 - 01/2004)

Related Diseases

1.	Anemia 11/01/2000 - "In a study of 232 individuals referred for molecular evaluation of anemia, 303 chromosomes carried alpha-globin gene abnormalities; 6 gene rearrangements and 11 point mutations were identified. " 09/01/1988 - "In addition, these studies suggest that small decreases in the amount of excess alpha-globin chains can significantly ameliorate the severity of anemia in the beta-thalassemic mouse." 08/01/2008 - "Excess alpha-globin precipitates in erythroid progenitor cells resulting in cell death, ineffective erythropoiesis and severe anemia. " 07/01/2007 - "Thus, compound mutants lacking both Ahsp and 1 of 4 alpha-globin genes (genotype Ahsp(-/-)alpha-globin*(alpha/alphaalpha)) exhibited more severe anemia and Hb instability than mice with either mutation alone. " 01/01/2006 - "The rationale behind this approach is to pair the excess normal alpha-globin chain with the alternative fetal gamma-chain to promote red blood cell survival and ameliorate the anemia. "
2.	beta-Thalassemia (Cooley's Anemia) 03/01/2010 - "This study revealed that all the three reported loci and the alpha-globin gene locus are the best and common predictors of the disease severity in beta-thalassemia." 06/01/2009 - "To study the effect of liposomal transfection of antisense oligonucleotide (ASON) on the erythroid cell alpha-globin gene in the patients with severe beta-thalassemia, and provide a new idea for beta-thalassemia gene therapy. " 10/01/2007 - "The objective of study was to investigate the effect of liposomal transfection of antisense oligodeoxynucleotide (ASON) on alpha-globin gene expression and proliferation of K562 cells, to explore the new way of gene therapy in beta-thalassemia. " 04/01/2006 - "There are few studies investigating alpha globin gene triplications in beta-thalassemia in Asian Indians and its effect on phenotype, which was the primary aim of this study. " 07/01/2004 - "In this study, the frequency of alpha globin gene deletions was studied in the beta-thalassemia trait, the mildest form of the disorder. "
3.	Hemolysis 09/01/2009 - "The excessive unbound alpha-globin chains precipitate in erythroid precursors and mature red blood cells leading to ineffective erythropoiesis and hemolysis. " 01/01/2008 - "This is predicted to result in precipitation of the unstable alpha chains or Hb variant, a concomitant decrease in the overall quantity of normal alpha-globin in the red cells and a potential degree of anemia and possibly, hemolysis. " 07/01/1972 - "Equal synthesis is probably due both to a decrease in alpha-chain synthesis and an increase in beta-chain synthesis in bone marrow erythroid cells and may contribute to the absence of overt hemolysis due to excess alpha-globin chain accumulation in heterozygous beta-thalassemia." 05/01/1999 - "It is concluded that the coinheritance of microdeletions in the alpha-globin gene locus in SSA patients confers "renoprotection" by mechanisms not related to the degree of anemia or the severity of hemolysis, but could be related to a reduced mean corpuscular volume or to a lower erythrocyte hemoglobin concentration." 05/01/2006 - "The results revealed that one of the possible molecular mechanism of the effects caused by Yisui Shengxue Granules is that it can up-regulate the expression levels of AHSP and erythroid transcription factor GATA-1 mRNAs, enhance the protein synthesis of AHSP which can bind the relative excess free alpha-globin, prevent the formation of alpha -globin-cytotoxic precipitates in red blood cells and decrease the hemolysis."
4.	Splenomegaly 04/01/1998 - "However, splenomegaly was more common as compared to the patients with a normal alpha-globin genotype. " 02/01/1988 - "The frequency of splenomegaly was not influenced by the alpha globin genotype. " 10/29/2002 - "Free alpha globin chains were completely cleared from red blood cell membranes, splenomegaly abated, and iron deposit was almost eliminated in liver sections. " 06/01/2003 - "Information, prospectively collected, included molecular defects, donor genotype, beta/alpha-globin expression rates, serum ferritin levels, hepato-splenomegaly, chelation history, virology screening, liver pathology together with post-transplant outcome inclusive of leucocyte chimerism. " 05/01/1987 - "Five patients with heterozygous beta-thalassaemia with an unusually severe clinical picture, low haemoglobin levels occasionally requiring blood transfusion, splenomegaly and unusually prominent basophilic stippling were found to have co-inherited a triple alpha-globin gene arrangement on one chromosome (alpha alpha alpha/alpha alpha). "
5.	Preleukemia 04/01/1987 - "The loss of alpha-globin gene transcriptional activity rarely occurs as an acquired abnormality during the evolution of myeloproliferative disease or preleukemia. " 10/01/1983 - "Acquired alpha-thalassemia in preleukemia is due to decreased expression of all four alpha-globin genes." 10/01/1983 - "A somatic mutation(s), acquired during the evolution of preleukemia in a 75-year-old Caucasian male of North European origin, resulted in a marked decrease in alpha-globin mRNA. " 08/01/1983 - "The presence of hemoglobin H (beta 4), resulting from a deficiency of alpha-globin chain synthesis, was observed as an acquired characteristic in the red cells of five elderly patients with myeloproliferative disorders or preleukemia. " 04/01/1987 - "These data showed that factor(s) in the mouse erythroleukemia cell can genetically complement the alpha-globin gene defect in these preleukemia patients with acquired hemoglobin H disease and suggest that altered expression of a gene in trans to the alpha-globin gene may be responsible for the acquisition of hemoglobin H disease in these patients."

Related Drugs and Biologics

1.	Messenger RNA (mRNA)
2.	Globins
3.	gamma-Globins
4.	Fetal Hemoglobin (Hemoglobin F)
5.	Iron
6.	RNA (Ribonucleic Acid)
7.	beta-Globins (beta Globin)
8.	DNA Restriction Enzymes (Restriction Endonuclease)
9.	Hemoglobins (Hemoglobin)
10.	DNA (Deoxyribonucleic Acid)

Related Therapies and Procedures

1.	Transplants (Transplant)
2.	Ligation
3.	Blood Transfusion (Blood Transfusions)