title: The expression of a truncated HMGI-C gene induces gigantism associated with lipomatosis.
abstract: Rearrangements of the HMGI-C gene have frequently been detected in human benign tumors of mesenchymal origin, including lipomas. The HMGI-C protein has three AT-hook domains and an acidic COOH-terminal tail. The HMGI-C modifications consist in the loss of the C-tail and the fusion with ectopic sequences. Recent results show that the loss of the COOH-terminal region, rather than the acquisition of new sequences, is sufficient to confer to HMGI-C the ability to transform NIH3T3 cells. Therefore, transgenic mice carrying a HMGI-C construct (HMGI-C/T), containing only the three AT-hook domains, were generated. The HMGI-C/T mice showed a giant phenotype, together with a predominantly abdominal/pelvic lipomatosis, suggesting a pivotal role of the HMGI-C truncation in the generation of human lipomas.
authors.affiliation: Kimmel Cancer Center, Jefferson Medical College, Philadelphia, Pennsylvania 19107, USA.
authors.forename: S
authors.initials: S
authors.lastname: Battista
authors.forename: V
authors.initials: V
authors.lastname: Fidanza
authors.forename: M
authors.initials: M
authors.lastname: Fedele
authors.forename: A J
authors.initials: AJ
authors.lastname: Klein-Szanto
authors.forename: E
authors.initials: E
authors.lastname: Outwater
authors.forename: H
authors.initials: H
authors.lastname: Brunner
authors.forename: M
authors.initials: M
authors.lastname: Santoro
authors.forename: C M
authors.initials: CM
authors.lastname: Croce
authors.forename: A
authors.initials: A
authors.lastname: Fusco
mesh_terms: D016475:3T3 Cells; D000273:Adipose Tissue / Q000033:anatomy & histology / Q000473:pathology; D000818:Animals; D002678:Chimera; D015321:Gene Rearrangement; D005877:Gigantism / Q000235:genetics* / Q000473:pathology; D025743:HMGA2 Protein; D006609:High Mobility Group Proteins / Q000096:biosynthesis / Q000235:genetics* / Q000378:metabolism*; D006801:Humans; D008068:Lipomatosis / Q000235:genetics* / Q000473:pathology; D008297:Male; D051379:Mice; D008822:Mice, Transgenic; D009363:Neoplasm Proteins / Q000235:genetics / Q000378:metabolism; D011994:Recombinant Proteins / Q000096:biosynthesis; D017384:Sequence Deletion; D013737:Testis / Q000033:anatomy & histology; D014162:Transfection; D001743:Urinary Bladder / Q000033:anatomy & histology
chemical_list: D025743:HMGA2 Protein; D006609:High Mobility Group Proteins; D009363:Neoplasm Proteins; D011994:Recombinant Proteins