Product Details

Purity

>85% (SDS-PAGE)

Target Names

Flcn

Uniprot No.

Q8QZS3

Alternative Names

FlcnFolliculin

Species

Mus musculus (Mouse)

Expression Region

1-579

Target Protein Sequence

MNAIVALCHF CELHGPRTLF CTEVLHAPLP QGAGSGDSPD QVEQAEEEEG GIQMSSRVRA HSPAEGASSE SSSPGPKKSD MCEGCRSLAV GHPGYISHDK ETSIKYVSHQ HPNHPQLFSI VRQACVRSLS CEVCPGREGP IFFGDEQHGF VFSHTFFIKD SLARGFQRWY SIIAIMMDRI YLINSWPFLL GRIRGIISEL QAKAFKVFEA EQFGCPQRAQ RMNTAFTPFL HQRNGNAARS LTSLTSDDNL WACLHTSFAW LLKACGSRLT EKLLEGAPTE DTLVQMEKLA DLEEESESWD NSEAEEEEKA PVTPEGAEGR ELTSCPTESS FLSACGSWQP PKLTGFKSLR HMRQVLGAPS FRMLAWHVLM GNQVIWKSRD VNLVHSAFEV LRTMLPVGCV RIIPYSSQYE EAYRCNFLGL SPPVPIPAHV LASEFVVVVE VHTATRSNLH PAGCEDDQSL SKYEFVVTSG SPVAADRVGP TILNKIEAAL TNQNLSVDVV DQCLICLKEE WMNKVKVLFK FTKVDSRPKE DTQKLLSVLG ASEEDNVKLL KFWMTGLSKT YKSHLMSTVR SPTATESRS

Protein Length

full length protein

Tag Info

Tag type will be determined during the manufacturing process.

The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.

Form

Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.

Buffer before Lyophilization

Tris/PBS-based buffer, 6% Trehalose, pH 8.0

Reconstitution

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Troubleshooting and FAQs

Protein FAQs

Storage Condition

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.

Shelf Life

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.

Lead Time

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.

Notes

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.

Datasheet

Please contact us to get it.

Target Background

Function

GTPase-activating protein that plays a key role in the cellular response to amino acid availability through regulation of the mTORC1 signaling cascade controlling the MiT/TFE factors TFEB and TFE3. Activates mTORC1 by acting as a GTPase-activating protein: specifically stimulates GTP hydrolysis by RRAGC/RagC or RRAGD/RagD, promoting the conversion to the GDP-bound state of RRAGC/RagC or RRAGD/RagD, and thereby activating the kinase activity of mTORC1. The GTPase-activating activity is inhibited during starvation and activated in presence of nutrients. Acts as a key component for mTORC1-dependent control of the MiT/TFE factors TFEB and TFE3, while it is not involved in mTORC1-dependent phosphorylation of canonical RPS6KB1/S6K1 and EIF4EBP1/4E-BP1. In low-amino acid conditions, the lysosomal folliculin complex (LFC) is formed on the membrane of lysosomes, which inhibits the GTPase-activating activity of FLCN, inactivates mTORC1 and maximizes nuclear translocation of TFEB and TFE3. Upon amino acid restimulation, RRAGA/RagA (or RRAGB/RagB) nucleotide exchange promotes disassembly of the LFC complex and liberates the GTPase-activating activity of FLCN, leading to activation of mTORC1 and subsequent cytoplasmic retention of TFEB and TFE3. Indirectly acts as a positive regulator of Wnt signaling by promoting mTOR-dependent cytoplasmic retention of MiT/TFE factor TFE3. Required for the exit of hematopoietic stem cell from pluripotency by promoting mTOR-dependent cytoplasmic retention of TFE3, thereby increasing Wnt signaling. Acts as an inhibitor of browning of adipose tissue by regulating mTOR-dependent cytoplasmic retention of TFE3. In response to flow stress, regulates STK11/LKB1 accumulation and mTORC1 activation through primary cilia: may act by recruiting STK11/LKB1 to primary cilia for activation of AMPK resided at basal bodies, causing mTORC1 down-regulation. Together with FNIP1 and/or FNIP2, regulates autophagy: following phosphorylation by ULK1, interacts with GABARAP and promotes autophagy. Required for starvation-induced perinuclear clustering of lysosomes by promoting association of RILP with its effector RAB34.

Gene References into Functions

Based on the phenotypes of our preclinical models, the FLCN H255Y mutant protein has lost it tumour suppressive function leading to the clinical manifestations of BHD, whereas the FLCN K508R mutant protein may have a dominant negative effect on the function of wild-type FLCN in regulating kidney cell proliferation and, therefore, act as an oncoprotein PMID: 28007907
FLCN regulates adipose tissue browning via mTOR and the transcription factor TFE3 PMID: 27913603
loss of FLCN results in a complete metabolic reprogramming of adipose tissues, resulting in enhanced oxidative metabolism. PMID: 27151976
mTOR inhibitor, sirolimus, suppresses the tumor's growth, suggesting that mTOR inhibitors might be effective in control of FLCN-deficient RCC. PMID: 26418749
we show that glycogen accumulates in kidneys from mice lacking FLCN and in renal tumors from a BHD patient PMID: 26439621
Folliculin (Flcn) inactivation leads to murine cardiac hypertrophy through mTORC1 deregulation. PMID: 24908670
The FLCN-GABARAP association is modulated by the presence of either folliculin-interacting protein (FNIP)-1 or FNIP2 and further regulated by ULK1. PMID: 25126726
Flcn regulates apoptosis in lung epithelium via E-cadherin-LKB1-AMPK axis. PMID: 24726356
loss of FLCN constitutively activates AMPK, resulting in PGC-1alpha-mediated mitochondrial biogenesis and increased ROS production PMID: 24762438
These data support a model in which dysregulation of the FLCN-p0071 interaction leads to alterations in cell adhesion, cell polarity, and RhoA signaling. PMID: 23139756
FLCN deficiency and subsequent increased PPARGC1A expression result in increased mitochondrial function and oxidative metabolism as the source of cellular energy, which may drive hyperplastic transformation. PMID: 23150719
Birt-Hogg-Dube (BHD) protein-deficient cells exhibited defects in cell-intrinsic apoptosis that correlated with reduced expression of the BH3-only protein Bim, which was similarly observed in all human and mouse BHD-related tumors examined. PMID: 21258407
FLCN is shown as a general tumour suppressor in the kidney. PMID: 19843504
Homozygous loss of BHD may initiate renal tumorigenesis in the mouse. The conditional BHD knockout mouse may be a useful research model for dissecting multistep kidney carcinogenesis, and rapamycin may be a potential treatment for Birt-Hogg-Dube syndrome PMID: 18182616
Deficiency of FLCN in mouse kidney led to development of polycystic kidneys and renal neoplasia. PMID: 18974783
These data support a role for PI3K-AKT pathway activation in kidney tumor formation caused by loss of BHD. PMID: 19850877

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Subcellular Location

Lysosome membrane. Cytoplasm, cytosol. Cell projection, cilium. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Cytoplasm, cytoskeleton, spindle. Nucleus.

Protein Families

Folliculin family

Tissue Specificity

Highly expressed in adult heart, pancreas, and prostate with moderate expression in adult brain, kidney, liver, adipose tissue and lung.

Database Links

KEGG: mmu:216805

STRING: 10090.ENSMUSP00000091696

UniGene: Mm.339640

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Recombinant Mouse Folliculin (Flcn)

Product Details

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Target Background

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