title: Matrix extracellular phosphoglycoprotein is expressed in causative tumors of oncogenic osteomalacia.
abstract: Oncogenic osteomalacia (OOM), or tumor-induced osteomalacia, is a rare disease characterized by renal phosphate wasting and osteomalacia. It arises due to the secretion of fibroblast growth factor 23 (FGF-23) from causative tumors. Matrix extracellular phosphoglycoprotein (MEPE) is predominantly expressed in odontoblasts, osteoblasts, and osteocytes. Although the presence of MEPE mRNA has been reported in some OOM tumors, little is known about the prevalence of MEPE expression in OOM tumors. In this study, the expression of MEPE and FGF-23 in OOM tumors was investigated at the transcriptional and translational levels. Eleven causative OOM tumors were analyzed by quantitative real-time reverse transcription-polymerase chain reaction and immunohistochemistry for MEPE and FGF-23 expression. Hemangiopericytomas and giant cell tumors, pathological diagnoses that are common in cases of OOM, were obtained from non-osteomalacic patients and analyzed as controls. The gene expression level of FGF23 and MEPE in OOM tumors was 10(4)- and 10(5)-times higher, respectively, than in non-OOM tumors. Immunohistochemical staining revealed that FGF-23 protein was expressed in all OOM tumors, and MEPE was expressed in 10 out of 11 OOM tumors. Thus, MEPE expression was common in OOM tumors, similar to FGF-23. These results indicate that, in addition to the hypophosphatemic effects of FGF-23, MEPE or the MEPE-derived acidic serine aspartate-rich MEPE-associated motif peptide may contribute to decreased bone mineralization in OOM patients.
authors: Department of Metabolism, Endocrinology and Molecular Medicine, Osaka City University Graduate School of Medicine, 1-4-3, Asahi-machi, Abeno-ku, Osaka 545-8585, Japan. imanishi@med.osaka-cu.ac.jp
authors: Yasuo
authors: Y
authors: Imanishi
authors: Jun
authors: J
authors: Hashimoto
authors: Wataru
authors: W
authors: Ando
authors: Keisuke
authors: K
authors: Kobayashi
authors: Takafumi
authors: T
authors: Ueda
authors: Yuki
authors: Y
authors: Nagata
authors: Akimitsu
authors: A
authors: Miyauchi
authors: Hajime M
authors: HM
authors: Koyano
authors: Hiroshi
authors: H
authors: Kaji
authors: Takatoshi
authors: T
authors: Saito
authors: Koichi
authors: K
authors: Oba
authors: Yasato
authors: Y
authors: Komatsu
authors: Tomoaki
authors: T
authors: Morioka
authors: Katsuhito
authors: K
authors: Mori
authors: Takami
authors: T
authors: Miki
authors: Masaaki
authors: M
authors: Inaba
mesh_terms: D000328:Adult; D000368:Aged; D016326:Extracellular Matrix Proteins / Q000235:genetics / Q000378:metabolism*; D005260:Female; D000089703:Fibroblast Growth Factor-23; D005346:Fibroblast Growth Factors / Q000235:genetics / Q000378:metabolism; D015972:Gene Expression Regulation, Neoplastic; D006023:Glycoproteins / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D009372:Neoplasms, Connective Tissue / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D010018:Osteomalacia; D010257:Paraneoplastic Syndromes; D010750:Phosphoproteins / Q000235:genetics / Q000378:metabolism*; D055815:Young Adult
chemical_list: D016326:Extracellular Matrix Proteins; C000717427:FGF23 protein, human; D006023:Glycoproteins; C486579:MEPE protein, human; D010750:Phosphoproteins; D005346:Fibroblast Growth Factors; D000089703:Fibroblast Growth Factor-23