GeneID: 36


Common Name: AHSP Type: Gene
Chromosome: 16 (NC_000016.10) Locus: NT_010393.16 (AHSP)
HUGO Symbol: AHSP Full Name: alpha hemoglobin stabilizing protein
Exons: 3 Introns: 2

AHSP is an abundant erythroid-specific protein that forms a stable complex with free alpha-globin but not beta-globin or the heterotetrameric haemoglobin A (α2β2). Moreover, AHSP specifically protects free alpha-globin from precipitating in solution and live cells. AHSP knock‐out mice show ineffective erythropoiesis and pathological features similar to those of β‐thalassaemia and loss of AHSP exacerbates the haematological abnormalities in β‐thalassaemic mice. AHSP is highly expressed in erythroid precursors and its expression is most abundant during late‐stage erythroid maturation. AHSP gene dosage would modulate pathologic states of alpha-globin excess such as beta-thalassaemia. The expression of AHSP gene has been observed to be controlled by a number of transcription factors including GATA-1, Oct-1, EKLF, STAT3 and NFE2.

Synonyms: EDRF, ERAF


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Publications / Origin

  1. Kihm AJ, Kong Y, Hong W, Russell JE, Rouda S, Adachi K, Simon MC, Blobel GA, Weiss MJ, An abundant erythroid protein that stabilizes free alpha-haemoglobin., Nature , 417(6890), 758-63, 2002 PubMed
  2. Kong Y, Zhou S, Kihm AJ, Katein AM, Yu X, Gell DA, Mackay JP, Adachi K, Foster-Brown L, Louden CS, Gow AJ, Weiss MJ, Loss of alpha-hemoglobin-stabilizing protein impairs erythropoiesis and exacerbates beta-thalassemia., J. Clin. Invest., 114(10), 1457-66, 2004 PubMed
  3. Feng L, Zhou S, Gu L, Gell DA, Mackay JP, Weiss MJ, Gow AJ, Shi Y, Structure of oxidized alpha-haemoglobin bound to AHSP reveals a protective mechanism for haem., Nature , 435(7042), 697-701, 2005 PubMed
  4. Che Yaacob NS, Islam MA, Alsaleh H, Ibrahim IK, Hassan R, Alpha-hemoglobin-stabilizing protein (AHSP): a modulatory factor in β-thalassemia., Int. J. Hematol., 111(3), 352-359, 2020 PubMed
Created on 2014-04-01 14:19:10, Last reviewed on 2020-06-29 13:57:59 (Show full history)

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