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Titolo:
Chromosome 16 loss of heterozygosity in tuberous sclerosis and sporadic lymphangiomyomatosis
Autore:
Yu, J; Astrinidis, A; Henske, EP;
Indirizzi:
Fox Chase Canc Ctr, Dept Med Oncol, Philadelphia, PA 19111 USA Fox Chase Canc Ctr Philadelphia PA USA 19111 , Philadelphia, PA 19111 USA
Titolo Testata:
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
fascicolo: 8, volume: 164, anno: 2001,
pagine: 1537 - 1540
SICI:
1073-449X(20011015)164:8<1537:C1LOHI>2.0.ZU;2-V
Fonte:
ISI
Lingua:
ENG
Soggetto:
CELL-ADHESION; TSC2 MUTATION; LYMPHANGIOLEIOMYOMATOSIS; GENE; RHO; HAMARTIN; ANGIOMYOLIPOMAS; MIGRATION; PROTEINS; COMPLEX;
Keywords:
lymphangiomyomatosis; tuberous sclerosis; genes; suppressor; tumor; loss of heterozygosity; chromosomes; human; pair 16;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
31
Recensione:
Indirizzi per estratti:
Indirizzo: Henske, EP Fox Chase Canc Ctr, Dept Med Oncol, 7701 Burholme Ave, Philadelphia, PA 19111 USA Fox Chase Canc Ctr 7701 Burholme Ave Philadelphia PA USA19111
Citazione:
J. Yu et al., "Chromosome 16 loss of heterozygosity in tuberous sclerosis and sporadic lymphangiomyomatosis", AM J R CRIT, 164(8), 2001, pp. 1537-1540

Abstract

In previous work we found loss of heterozygosity (LOH) of the wild-type TSC2 allele in the abnormal pulmonary smooth muscle cells and renal angiomyolipoma cells from patients with sporadic pulmonary lymphangiomyomatosis (LAM). Here we report TSC2 LOH in microdissected pulmonary LAM cells from a patient with tuberous sclerosis complex (TSC), demonstrating for the first time that the two-hit tumor suppressor gene model applies to the TSC-associated, as well as sporadic LAM. We also compared the chromosome 16 LOH region between angiomyolipoma and pulmonary LAM from two patients with sporadic LAM. Previously we found that these patients had TSC2 mutations and TSC2 LOH in their angiomyolipomas and pulmonary LAM cells but not in normal lung or kidney cells. This suggests that pulmonary LAM may result from the migrationof smooth muscle cells from renal angiomyolipomas to the lung. In this case, one would predict that the angiomyolipoma and LAM cells would have identical LOH patterns. We found that at each chromosome 16 marker, the results were concordant between angiomyolipoma and LAM. This is consistent with a model in which pulmonary LAM cells and angiomyolipoma cells have a common genetic origin.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/04/20 alle ore 12:09:50