TY - JOUR
T1 - Spectrin isoforms
T2 - Differential expression in normal hematopoiesis and alterations in neoplastic bone marrow disorders
AU - Wolgast, Lucia R.
AU - Cannizzarro, Linda A.
AU - Ramesh, K. H.
AU - Xue, Xiaonan
AU - Wang, Dan
AU - Bhattacharyya, Pritish K.
AU - Gong, Jerald Z.
AU - McMahon, Christine
AU - Albanese, Joseph M.
AU - Sunkara, Jaya L.
AU - Ratech, Howard
PY - 2011/8
Y1 - 2011/8
N2 - 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.
AB - 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.
KW - Acute erythroid leukemia
KW - Acute megakaryoblastic leukemia
KW - Acute myeloid leukemia
KW - Embryonic liver fodrin
KW - Myelodysplastic syndrome
KW - Myeloproliferative neoplasm
KW - Spectrin
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U2 - 10.1309/AJCPSA5RNM9IGFJF
DO - 10.1309/AJCPSA5RNM9IGFJF
M3 - Article
C2 - 21757604
AN - SCOPUS:79960989837
SN - 0002-9173
VL - 136
SP - 300
EP - 308
JO - American journal of clinical pathology
JF - American journal of clinical pathology
IS - 2
ER -