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Fusion of ALK to the Ran-binding protein 2 (RANBP2) gene in inflammatory myofibroblastic tumor.
Genes Chromosomes Cancer 2003; 37(1):98-105GC

Abstract

Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal proliferation of transformed myofibroblasts, with a prominent inflammatory cell component, that can mimic other spindle cell processes such as nodular fasciitis, desmoid tumor, and gastrointestinal stromal tumor. Genetic analyses have recently demonstrated rearrangements of anaplastic lymphoma kinase (ALK), located at 2p23, in a subset of IMTs. Molecular characterizations have identified ALK fusions involving tropomyosin-3 and -4 (TPM-3 and -4), the clathrin heavy chain (CLTC), and the cysteinyl-tRNA synthetase (CARS) genes as fusion partners. Here we describe two IMTs with a novel ALK fusion that involves the Ran-binding protein 2 (RANBP2) gene at 2q13, which normally encodes a large (358-kDa) nucleopore protein localized at the cytoplasmic side of the nuclear pore complex. The N-terminal 867 residues of RANBP2 are fused to the cytoplasmic segment of ALK in the 1,430-amino acid RANBP2-ALK chimeric protein. Myofibroblasts that express RANBP2-ALK exhibit nuclear membrane-associated ALK staining that is unique compared to the subcellular localization observed with other ALK fusions in IMT, presumably attributable to heteroassociation of the fusion with normal RANBP2 at the nuclear pore. These findings expand the spectrum of ALK abnormalities observed in IMT and further confirm the clonal, neoplastic nature of these lesions.

Authors+Show Affiliations

Department of Pathology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105-2794, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

12661011

Citation

Ma, Zhigui, et al. "Fusion of ALK to the Ran-binding Protein 2 (RANBP2) Gene in Inflammatory Myofibroblastic Tumor." Genes, Chromosomes & Cancer, vol. 37, no. 1, 2003, pp. 98-105.
Ma Z, Hill DA, Collins MH, et al. Fusion of ALK to the Ran-binding protein 2 (RANBP2) gene in inflammatory myofibroblastic tumor. Genes Chromosomes Cancer. 2003;37(1):98-105.
Ma, Z., Hill, D. A., Collins, M. H., Morris, S. W., Sumegi, J., Zhou, M., ... Bridge, J. A. (2003). Fusion of ALK to the Ran-binding protein 2 (RANBP2) gene in inflammatory myofibroblastic tumor. Genes, Chromosomes & Cancer, 37(1), pp. 98-105.
Ma Z, et al. Fusion of ALK to the Ran-binding Protein 2 (RANBP2) Gene in Inflammatory Myofibroblastic Tumor. Genes Chromosomes Cancer. 2003;37(1):98-105. PubMed PMID: 12661011.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Fusion of ALK to the Ran-binding protein 2 (RANBP2) gene in inflammatory myofibroblastic tumor. AU - Ma,Zhigui, AU - Hill,D Ashley, AU - Collins,Margaret H, AU - Morris,Stephan W, AU - Sumegi,Janos, AU - Zhou,Ming, AU - Zuppan,Craig, AU - Bridge,Julia A, PY - 2003/3/28/pubmed PY - 2003/5/13/medline PY - 2003/3/28/entrez SP - 98 EP - 105 JF - Genes, chromosomes & cancer JO - Genes Chromosomes Cancer VL - 37 IS - 1 N2 - Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal proliferation of transformed myofibroblasts, with a prominent inflammatory cell component, that can mimic other spindle cell processes such as nodular fasciitis, desmoid tumor, and gastrointestinal stromal tumor. Genetic analyses have recently demonstrated rearrangements of anaplastic lymphoma kinase (ALK), located at 2p23, in a subset of IMTs. Molecular characterizations have identified ALK fusions involving tropomyosin-3 and -4 (TPM-3 and -4), the clathrin heavy chain (CLTC), and the cysteinyl-tRNA synthetase (CARS) genes as fusion partners. Here we describe two IMTs with a novel ALK fusion that involves the Ran-binding protein 2 (RANBP2) gene at 2q13, which normally encodes a large (358-kDa) nucleopore protein localized at the cytoplasmic side of the nuclear pore complex. The N-terminal 867 residues of RANBP2 are fused to the cytoplasmic segment of ALK in the 1,430-amino acid RANBP2-ALK chimeric protein. Myofibroblasts that express RANBP2-ALK exhibit nuclear membrane-associated ALK staining that is unique compared to the subcellular localization observed with other ALK fusions in IMT, presumably attributable to heteroassociation of the fusion with normal RANBP2 at the nuclear pore. These findings expand the spectrum of ALK abnormalities observed in IMT and further confirm the clonal, neoplastic nature of these lesions. SN - 1045-2257 UR - https://www.unboundmedicine.com/medline/citation/12661011/Fusion_of_ALK_to_the_Ran_binding_protein_2__RANBP2__gene_in_inflammatory_myofibroblastic_tumor_ L2 - https://doi.org/10.1002/gcc.10177 DB - PRIME DP - Unbound Medicine ER -