A germ-line insertion in the Birt-Hogg-Dubé (BHD) gene gives rise to the Nihon rat model of inherited renal cancer
about
Serine 62 is a phosphorylation site in folliculin, the Birt-Hogg-Dubé gene productInteraction of folliculin (Birt-Hogg-Dubé gene product) with a novel Fnip1-like (FnipL/Fnip2) proteinInactivation of the FLCN tumor suppressor gene induces TFE3 transcriptional activity by increasing its nuclear localizationTumor suppressor FLCN inhibits tumorigenesis of a FLCN-null renal cancer cell line and regulates expression of key molecules in TGF-beta signalingBirt-Hogg-Dube syndrome: clinicopathological features of the lungBHD mutations, clinical and molecular genetic investigations of Birt-Hogg-Dubé syndrome: a new series of 50 families and a review of published reportsExpression and knockdown of zebrafish folliculin suggests requirement for embryonic brain morphogenesis.Homozygous loss of BHD causes early embryonic lethality and kidney tumor development with activation of mTORC1 and mTORC2Kidney-targeted Birt-Hogg-Dube gene inactivation in a mouse model: Erk1/2 and Akt-mTOR activation, cell hyperproliferation, and polycystic kidneysDeficiency of FLCN in mouse kidney led to development of polycystic kidneys and renal neoplasiaThe role of the Birt-Hogg-Dubé protein in mTOR activation and renal tumorigenesisLoss of the Birt-Hogg-Dubé tumor suppressor results in apoptotic resistance due to aberrant TGFβ-mediated transcriptionSpontaneous renal tubular hyperplastic and neoplastic lesions in three Sprague-Dawley rats from a 90-day toxicity study.A 4-bp deletion in the Birt-Hogg-Dubé gene (FLCN) causes dominantly inherited spontaneous pneumothorax.Germline BHD-mutation spectrum and phenotype analysis of a large cohort of families with Birt-Hogg-Dubé syndromeFolliculin regulates cell-cell adhesion, AMPK, and mTORC1 in a cell-type-specific manner in lung-derived cells.The folliculin mutation database: an online database of mutations associated with Birt-Hogg-Dubé syndromeLung cysts, spontaneous pneumothorax, and genetic associations in 89 families with Birt-Hogg-Dubé syndromeFLCN Maintains the Leucine Level in Lysosome to Stimulate mTORC1.Renal oncocytosis.Molecular genetics and clinical features of Birt-Hogg-Dubé syndrome.Birt-Hogg-Dubé syndrome: Clinical and molecular aspects of recently identified kidney cancer syndrome.The Drosophila homolog of the human tumor suppressor gene BHD interacts with the JAK-STAT and Dpp signaling pathways in regulating male germline stem cell maintenance.FLCN: The causative gene for Birt-Hogg-Dubé syndrome.Early onset of spontaneous renal preneoplastic and neoplastic lesions in young conventional rats in toxicity studies.Transgenic rescue from embryonic lethality and renal carcinogenesis in the Nihon rat model by introduction of a wild-type Bhd gene.The Birt-Hogg-Dube and tuberous sclerosis complex homologs have opposing roles in amino acid homeostasis in Schizosaccharomyces pombe.
P2860
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P2860
A germ-line insertion in the Birt-Hogg-Dubé (BHD) gene gives rise to the Nihon rat model of inherited renal cancer
description
2004 nî lūn-bûn
@nan
2004年の論文
@ja
2004年論文
@yue
2004年論文
@zh-hant
2004年論文
@zh-hk
2004年論文
@zh-mo
2004年論文
@zh-tw
2004年论文
@wuu
2004年论文
@zh
2004年论文
@zh-cn
name
A germ-line insertion in the B ...... odel of inherited renal cancer
@ast
A germ-line insertion in the B ...... odel of inherited renal cancer
@en
type
label
A germ-line insertion in the B ...... odel of inherited renal cancer
@ast
A germ-line insertion in the B ...... odel of inherited renal cancer
@en
prefLabel
A germ-line insertion in the B ...... odel of inherited renal cancer
@ast
A germ-line insertion in the B ...... odel of inherited renal cancer
@en
P2093
P2860
P356
P1476
A germ-line insertion in the B ...... odel of inherited renal cancer
@en
P2093
Berton Zbar
Hiroaki Mitani
Junko Sakurai
Kazuo Okimoto
Laura S Schmidt
Michael L Nickerson
Michelle B Warren
Toshiyuki Kobayashi
Youko Hirayama
P2860
P304
P356
10.1073/PNAS.0308071100
P407
P577
2004-02-09T00:00:00Z