title: Spectrin isoforms: differential expression in normal hematopoiesis and alterations in neoplastic bone marrow disorders.
abstract: Spectrins are large, rod-like, multifunctional molecules that participate in maintaining cell structure, signal transmission, and DNA repair. Because little is known about the role of spectrins in normal hematopoiesis and leukemogenesis, we immunohistochemically stained bone marrow biopsy specimens from 81 patients for αI, αII, βI, and βII spectrin isoforms in normal reactive marrow (NRM), myelodysplastic syndrome, myeloproliferative neoplasm, acute myeloid leukemia (AML) with well-characterized cytogenetic abnormalities, acute erythroid leukemia (EryL), and acute megakaryoblastic leukemia (MegL). In NRM, spectrin isoforms were differentially expressed according to cell lineage: αI and βI in erythroid precursors; αII and βII in granulocytes; and βI and βII in megakaryocytes. In contrast, 18 (44%) of 41 AMLs lacked αII spectrin and/or aberrantly expressed βI spectrin (P = .0398; Fisher exact test) and 5 (100%) of 5 EryLs expressed βII spectrin but lacked βI spectrin. The frequent loss and/or gain of spectrin isoforms in AMLs suggests a possible role for spectrin in leukemogenesis.
authors.affiliation: Department of Pathology, Montefiore Medical Center, Bronx, NY 10467, USA.
authors.forename: Lucia R
authors.initials: LR
authors.lastname: Wolgast
authors.forename: Linda A
authors.initials: LA
authors.lastname: Cannizzarro
authors.forename: K H
authors.initials: KH
authors.lastname: Ramesh
authors.forename: Xiaonan
authors.initials: X
authors.lastname: Xue
authors.forename: Dan
authors.initials: D
authors.lastname: Wang
authors.forename: Pritish K
authors.initials: PK
authors.lastname: Bhattacharyya
authors.forename: Jerald Z
authors.initials: JZ
authors.lastname: Gong
authors.forename: Christine
authors.initials: C
authors.lastname: McMahon
authors.forename: Joseph M
authors.initials: JM
authors.lastname: Albanese
authors.forename: Jaya L
authors.initials: JL
authors.lastname: Sunkara
authors.forename: Howard
authors.initials: H
authors.lastname: Ratech
mesh_terms: D014408:Biomarkers, Tumor / Q000032:analysis*; D006410:Hematopoiesis / Q000502:physiology*; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D015470:Leukemia, Myeloid, Acute / Q000378:metabolism*; D009190:Myelodysplastic Syndromes / Q000378:metabolism* / Q000473:pathology; D009196:Myeloproliferative Disorders / Q000473:pathology; D020033:Protein Isoforms / Q000032:analysis / Q000096:biosynthesis; D012189:Retrospective Studies; D013049:Spectrin / Q000032:analysis / Q000096:biosynthesis*
chemical_list: D014408:Biomarkers, Tumor; D020033:Protein Isoforms; D013049:Spectrin