CURRENT CLINICAL INTERESTS
Benign red blood cell disorders, iron metabolism (including iron
deficiency and hereditary hemochromatosis), acquired aplastic anemia,
congenital marrow failure syndromes (including Diamond-Blackfan anemia
and Dyskeratosis Congenita) and the transition of care between
pediatric and adult hematologists, neurovisceral porphyrias
CURRENT RESEARCH INTERESTS
Normal and ineffective erythropoiesis, systemic and cellular iron homeostasis, and the clinical presentation and molecular underpinnings of inherited marrow failure.
The Keel Laboratory aims to understand normal and abnormal red blood cell development. Current work examines the molecular and cellular events in late-stage erythroid maturation and in particular the mechanisms that coordinate heme synthesis, iron availability, erythropoietin response, and cellular metabolism. A second project in the lab centers on inherited bone marrow failure and inherited myelodysplastic syndromes and aims to define their clinical phenotypes, natural histories, and underlying genetics to improve diagnostic accuracy and clinical care.
Keel SB, Abkowitz JL. The microcytic red cell and the anemia of
Engl J Med. 2009 Nov 5;361(19):1904-6. PMID: 19890136
Keel SB, Doty RT, Yang Z, Quigley JG, Chen J, Knoblaugh S, Kingsley PD, De
Domenico I, Vaughn MB, Kaplan J, Palis J, Abkowitz JL. A heme
export protein is required for red blood cell differentiation and iron
2008 Feb 8;319(5864):825-8. PMID: 18258918.
JG, Keel SB, Reyes M,
Burris SM. Alpha-delta storage deficiency in three
generations. Platelets. 2007
Keel SB, Abkowitz JL: Pure red cell aplasia. IN: Clinical Hematology, Young NS, Gerson SL, High KA (eds.), pp
169-180, Philadelphia: Elsevier, 2006.
Keel SB, Phelps S, Sabo KM, O'Leary MN, Kirn-Safran CB, Abkowitz JL. Establishing Rps6 hemizygous mice as a model for studying how ribosomal protein haploinsufficiency impairs erythropoiesis. Exp Hematol. 2012 Apr;40(4):290-4. PMID: 22198155.
Liu L, Sánchez-Bonilla M, Crouthamel M, Giachelli C, and Keel SB. Mice lacking the sodium-dependent phosphate import protein, PiT1 (SLC20A1), have a severe defect in terminal erythroid differentiation and early B-cell development. Experimental Hematology, 2013 May;41(5):432-43. PMID: 23376999.
Zhang MY1, Keel SB2, Walsh T2, Lee MK2, Gulsuner S2, Watts AC2, Pritchard CC2, Salipante SJ2, Jeng MR3, Hofmann I4, Williams DA4, Fleming MD4, Abkowitz JL2, King MC2, Shimamura A5. Genomic analysis of bone marrow failure and myelodysplastic syndromes reveals phenotypic and diagnostic complexity. Haematologica 2014 Sep 19. pii: haematol.2014.113456. PMID: 25239263.