Alpha-hemoglobin-stabilizing protein
Details
- Name
- Alpha-hemoglobin-stabilizing protein
- Synonyms
- EDRF
- ERAF
- Erythroid differentiation-related factor
- Erythroid-associated factor
- Gene Name
- AHSP
- Organism
- Humans
- Amino acid sequence
>lcl|BSEQ0049694|Alpha-hemoglobin-stabilizing protein MALLKANKDLISAGLKEFSVLLNQQVFNDPLVSEEDMVTVVEDWMNFYINYYRQQVTGEP QERDKALQELRQELNTLANPFLAKYRDFLKSHELPSHPPPSS
- Number of residues
- 102
- Molecular Weight
- 11840.325
- Theoretical pI
- Not Available
- GO Classification
- Functionshemoglobin binding / unfolded protein bindingProcesseserythrocyte differentiation / hemoglobin metabolic process / hemopoiesis / protein folding / protein stabilizationComponentshemoglobin complex
- General Function
- Acts as a chaperone to prevent the harmful aggregation of alpha-hemoglobin during normal erythroid cell development. Specifically protects free alpha-hemoglobin from precipitation. It is predicted to modulate pathological states of alpha-hemoglobin excess such as beta-thalassemia.
- Specific Function
- Hemoglobin binding
- Pfam Domain Function
- AHSP (PF09236)
- Transmembrane Regions
- Not Available
- Cellular Location
- Cytoplasm
- Gene sequence
>lcl|BSEQ0049695|Alpha-hemoglobin-stabilizing protein (AHSP) ATGGCTCTTCTTAAGGCCAATAAGGATCTCATTTCCGCAGGATTGAAGGAGTTCAGCGTT CTGCTGAATCAGCAGGTCTTCAATGATCCTCTCGTCTCTGAAGAAGACATGGTGACTGTG GTGGAGGACTGGATGAACTTCTACATCAACTATTACAGGCAGCAGGTGACAGGGGAGCCC CAAGAGCGAGACAAGGCTCTGCAGGAGCTTCGGCAAGAGCTGAACACTCTGGCCAACCCT TTCCTGGCCAAGTACAGGGACTTCCTGAAGTCTCATGAGCTCCCGAGTCACCCACCGCCC TCCTCCTAG
- Chromosome Location
- 16
- Locus
- 16p11.2
- External Identifiers
Resource Link UniProtKB ID Q9NZD4 UniProtKB Entry Name AHSP_HUMAN HGNC ID HGNC:18075 - General References
- Miele G, Manson J, Clinton M: A novel erythroid-specific marker of transmissible spongiform encephalopathies. Nat Med. 2001 Mar;7(3):361-4. [Article]
- Zhang QH, Ye M, Wu XY, Ren SX, Zhao M, Zhao CJ, Fu G, Shen Y, Fan HY, Lu G, Zhong M, Xu XR, Han ZG, Zhang JW, Tao J, Huang QH, Zhou J, Hu GX, Gu J, Chen SJ, Chen Z: Cloning and functional analysis of cDNAs with open reading frames for 300 previously undefined genes expressed in CD34+ hematopoietic stem/progenitor cells. Genome Res. 2000 Oct;10(10):1546-60. [Article]
- Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [Article]
- 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. 2002 Jun 13;417(6890):758-63. [Article]
- Gell D, Kong Y, Eaton SA, Weiss MJ, Mackay JP: Biophysical characterization of the alpha-globin binding protein alpha-hemoglobin stabilizing protein. J Biol Chem. 2002 Oct 25;277(43):40602-9. Epub 2002 Aug 20. [Article]
- Feng L, Gell DA, Zhou S, Gu L, Kong Y, Li J, Hu M, Yan N, Lee C, Rich AM, Armstrong RS, Lay PA, Gow AJ, Weiss MJ, Mackay JP, Shi Y: Molecular mechanism of AHSP-mediated stabilization of alpha-hemoglobin. Cell. 2004 Nov 24;119(5):629-40. [Article]
- Santiveri CM, Perez-Canadillas JM, Vadivelu MK, Allen MD, Rutherford TJ, Watkins NA, Bycroft M: NMR structure of the alpha-hemoglobin stabilizing protein: insights into conformational heterogeneity and binding. J Biol Chem. 2004 Aug 13;279(33):34963-70. Epub 2004 Jun 3. [Article]
- 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. 2005 Jun 2;435(7042):697-701. [Article]
Drug Relations
- Drug Relations
DrugBank ID Name Drug group Pharmacological action? Actions Details DB01592 Iron approved unknown Details DB14488 Ferrous gluconate approved unknown Details DB14489 Ferrous succinate approved unknown Details DB14490 Ferrous ascorbate approved unknown Details DB14491 Ferrous fumarate approved unknown Details DB14501 Ferrous glycine sulfate approved unknown Details