The folliculin-FNIP1 pathway deleted in human Birt-Hogg-Dubé syndrome is required for murine B-cell development
about
Discovery of Novel DENN Proteins: Implications for the Evolution of Eukaryotic Intracellular Membrane Structures and Human DiseaseFLCN, a novel autophagy component, interacts with GABARAP and is regulated by ULK1 phosphorylationThe product of C9orf72, a gene strongly implicated in neurodegeneration, is structurally related to DENN Rab-GEFsFLCN and AMPK Confer Resistance to Hyperosmotic Stress via Remodeling of Glycogen StoresFolliculin regulates ampk-dependent autophagy and metabolic stress survivalExit from pluripotency is gated by intracellular redistribution of the bHLH transcription factor Tfe3Metabolic regulator Fnip1 is crucial for iNKT lymphocyte development.Folliculin regulates cell-cell adhesion, AMPK, and mTORC1 in a cell-type-specific manner in lung-derived cells.Folliculin-interacting proteins Fnip1 and Fnip2 play critical roles in kidney tumor suppression in cooperation with Flcn.Clinical Features, Genetics and Potential Therapeutic Approaches for Birt-Hogg-Dubé Syndrome.Birt-Hogg-Dubé syndrome: from gene discovery to molecularly targeted therapies.Loss of Folliculin Disrupts Hematopoietic Stem Cell Quiescence and Homeostasis Resulting in Bone Marrow Failure.Tumor Suppressor Folliculin Regulates mTORC1 through Primary CiliaMutation of Fnip1 is associated with B-cell deficiency, cardiomyopathy, and elevated AMPK activity.Genetic modification of dividing cells using episomally maintained S/MAR DNA vectors.Ikaros is absolutely required for pre-B cell differentiation by attenuating IL-7 signalsThe folliculin tumor suppressor is a GAP for the RagC/D GTPases that signal amino acid levels to mTORC1.Molecular genetics and clinical features of Birt-Hogg-Dubé syndrome.Nutrient-induced FNIP degradation by SCFβ-TRCP regulates FLCN complex localization and promotes renal cancer progressionBirt-Hogg-Dubé syndrome: Clinical and molecular aspects of recently identified kidney cancer syndrome.Control of B lymphocyte development and functions by the mTOR signaling pathways.Suppression of autophagy enhances preferential toxicity of paclitaxel to folliculin-deficient renal cancer cellsLysosomal Regulation of mTORC1 by Amino Acids in Mammalian Cells.Glycogen: A must have storage to survive stressful emergenciesFLCN: The causative gene for Birt-Hogg-Dubé syndrome.Hereditary kidney cancer syndromes: Genetic disorders driven by alterations in metabolism and epigenome regulation.H255Y and K508R missense mutations in tumour suppressor folliculin (FLCN) promote kidney cell proliferation.A Brake for B Cell Proliferation: Appropriate responses to metabolic stress are crucial to maintain B cell viability and prevent malignant outgrowth.Loss of Fnip1 alters kidney developmental transcriptional program and synergizes with TSC1 loss to promote mTORC1 activation and renal cyst formation.
P2860
Q21131245-8E3FBC41-AE88-46C5-8923-E253205C80DFQ24302037-94B78946-D32F-4693-902C-9CD7A14A68D3Q24622048-DEDB0201-2F08-4F57-B504-14E651AB73E5Q27310260-3912AE93-0E2B-4DB8-97DA-048C726D1834Q27316476-2292B4FB-DB14-4040-B2AF-BE4790B94E3EQ28594750-060F9711-E45D-48DC-A50A-9A142D45EA7DQ33627305-40FE3684-FE54-4475-AE78-6A7D7E5776CDQ34582386-A33056E1-68CC-4529-A30C-AF09CC7BAB16Q35279496-CA0736A1-3D84-48B8-92CB-99BBAF750625Q36283086-9A2CB106-4049-4632-8469-4CC3E6EEE985Q36799958-6FB72CBE-1B90-4CD3-8E04-18174101AA64Q36832543-8323EC33-7765-400D-9E70-54117C9DBC01Q36941091-A872B07B-306A-48C0-A02E-84A794ADE366Q37065314-445A2306-4751-44F4-8BDF-A86F92E4B7ABQ37138704-125FA695-C59B-426C-A0E2-C0F179E804CDQ37397804-B5F1493A-D7B8-4A9D-8D57-C58D6CFCB560Q37402791-E9EC5A5A-475E-4EC2-AF7A-F80066A6C6BCQ37429054-1E3772B4-FC83-4FF5-A83A-62D26BF6B6D2Q37706087-FA31F4B5-E62D-414B-9E59-0505AA8820F4Q38647953-0FBBC2EC-9EEF-4310-9776-75ECF8452B14Q38674507-A669AB74-1580-4F75-92A5-9B110657A76FQ39048858-0C6AE9E0-EC93-40D2-BEA4-F54472E1DB3EQ39420775-17066A4C-EA89-4234-9EA6-DB7BECF61333Q39627855-8C72FA50-83D5-4D66-940E-62C5A4E3420EQ47712748-ACA2EB72-A5A2-49C8-8BAE-2FE6CDEBFEBCQ50083718-5464C414-011F-4806-8D6C-07D6F938F39BQ51241419-FDEB2663-791D-4D50-B2B6-C0CBBD8812A8Q53053529-B87B46A7-DC99-4307-854A-531403D46135Q55500213-7469497E-7F8A-4E31-BF48-382A56776BB2
P2860
The folliculin-FNIP1 pathway deleted in human Birt-Hogg-Dubé syndrome is required for murine B-cell development
description
2012 թուականի Օգոստոսին հրատարակուած գիտական յօդուած
@hyw
2012 թվականի օգոստոսին հրատարակված գիտական հոդված
@hy
artículu científicu espublizáu en 2012
@ast
im August 2012 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 2012/08/09)
@sk
vědecký článek publikovaný v roce 2012
@cs
wetenschappelijk artikel (gepubliceerd op 2012/08/09)
@nl
наукова стаття, опублікована в серпні 2012
@uk
مقالة علمية (نشرت في 9-8-2012)
@ar
name
The folliculin-FNIP1 pathway d ...... for murine B-cell development
@ast
The folliculin-FNIP1 pathway d ...... for murine B-cell development
@en
The folliculin-FNIP1 pathway d ...... for murine B-cell development
@nl
type
label
The folliculin-FNIP1 pathway d ...... for murine B-cell development
@ast
The folliculin-FNIP1 pathway d ...... for murine B-cell development
@en
The folliculin-FNIP1 pathway d ...... for murine B-cell development
@nl
prefLabel
The folliculin-FNIP1 pathway d ...... for murine B-cell development
@ast
The folliculin-FNIP1 pathway d ...... for murine B-cell development
@en
The folliculin-FNIP1 pathway d ...... for murine B-cell development
@nl
P2093
P2860
P50
P1433
P1476
The folliculin-FNIP1 pathway d ...... for murine B-cell development
@en
P2093
Carme Gallego Gonzalez
Eileen Southon
Hisashi Hasumi
Hong-Wei Sun
Hyoungbin F Oh
Jonathan R Keller
Kyong-Rim Kieffer-Kwon
Laura S Schmidt
Lino Tessarollo
Mara E Klein
P2860
P304
P356
10.1182/BLOOD-2012-02-410407
P407
P577
2012-06-18T00:00:00Z