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
Functions
hemoglobin binding / unfolded protein binding
Processes
erythrocyte differentiation / hemoglobin metabolic process / hemopoiesis / protein folding / protein stabilization
Components
hemoglobin 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
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
ResourceLink
UniProtKB IDQ9NZD4
UniProtKB Entry NameAHSP_HUMAN
HGNC IDHGNC:18075
General References
  1. Miele G, Manson J, Clinton M: A novel erythroid-specific marker of transmissible spongiform encephalopathies. Nat Med. 2001 Mar;7(3):361-4. [PubMed:11231637]
  2. 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. [PubMed:11042152]
  3. 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. [PubMed:15489334]
  4. 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. [PubMed:12066189]
  5. 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. [PubMed:12192002]
  6. 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. [PubMed:15550245]
  7. 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. [PubMed:15178680]
  8. 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. [PubMed:15931225]

Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB01592IronapprovedunknownDetails
DB14488Ferrous gluconateapprovedunknownDetails
DB14489Ferrous succinateapprovedunknownDetails
DB14490Ferrous ascorbateapprovedunknownDetails
DB14491Ferrous fumarateapprovedunknownDetails
DB14501Ferrous glycine sulfateapprovedunknownDetails