Anti-FLCN 抗体 [EPNCIR147] (ab124885)
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPNCIR147] to FLCN
- Suitable for: WB
- Knockout validated
- Reacts with: Human
Related conjugates and formulations
製品の概要
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製品名
Anti-FLCN antibody [EPNCIR147]
FLCN 一次抗体 製品一覧 -
製品の詳細
Rabbit monoclonal [EPNCIR147] to FLCN -
由来種
Rabbit -
アプリケーション
適用あり: WBmore details
適用なし: Flow Cyt,ICC/IF,IHC-P or IP -
種交差性
交差種: Human
交差が予測される動物種: Mouse, Rat -
免疫原
Recombinant fragment (GST-tag) corresponding to Human FLCN.
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ポジティブ・コントロール
- WB: Wild-type HeLa, HEK-293, HeLa, HEK-293T and NCCIT cell lysates.
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特記事項
This antibody was developed as part of a collaboration between the National Cancer Institute's Center for Cancer Research and the lab of Marston Linehan. View antibodies from NCI Center for Cancer Research Collaboration.
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
製品の特性
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製品の状態
Liquid -
保存方法
Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C. -
バッファー
pH: 7.2
Preservative: 0.05% Sodium azide
Constituents: 40% Glycerol (glycerin, glycerine), 9.85% Tris glycine, 50% Tissue culture supernatant -
Concentration information loading...
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精製度
Protein A purified -
ポリ/モノ
モノクローナル -
クローン名
EPNCIR147 -
アイソタイプ
IgG -
研究分野
関連製品
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Alternative Versions
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Isotype control
アプリケーション
The Abpromise guarantee
Abpromise保証は、 次のテスト済みアプリケーションにおけるab124885の使用に適用されます
アプリケーションノートには、推奨の開始希釈率がありますが、適切な希釈率につきましてはご検討ください。
アプリケーション | Abreviews | 特記事項 |
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WB |
1/1000 - 1/10000. Detects a band of approximately 70 kDa (predicted molecular weight: 64 kDa).
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特記事項 |
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WB
1/1000 - 1/10000. Detects a band of approximately 70 kDa (predicted molecular weight: 64 kDa). |
ターゲット情報
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機能
May play a role in the pathogenesis of an uncommon form of kidney cancer through its association with an inherited disorder of the hair follicle (fibrofolliculomas). May be a tumor suppressor. May be involved in colorectal tumorigenesis. May be involved in energy and/or nutrient sensing through the AMPK and mTOR signaling pathways. May regulate phosphorylation of RPS6KB1. -
組織特異性
Expressed in most tissues tested, including skin, lung, kidney, heart, testis and stomach. -
関連疾患
Defects in FLCN are the cause of Birt-Hogg-Dube syndrome (BHD) [MIM:135150]. BHD is a rare autosomal dominant genodermatosis characterized by hair follicle hamartomas (fibrofolliculomas), kidney tumors, and spontaneous pneumothorax. Fibrofolliculomas are part of the triad of BHD skin lesions that also includes trichodiscomas and acrochordons. Onset of this dermatologic condition is invariably in adulthood. BHD is associated with a variety of histologic types of renal tumors, including chromophobe renal cell carcinoma (RCC), benign renal oncocytoma, clear-cell RCC and papillary type I RCC. Multiple lipomas, angiolipomas, and parathyroid adenomas are also seen in patients affected with this disease. The majority of mutations are predicted to prematurely terminate the protein.
Defects in FLCN are in some cases a cause of primary spontaneous pneumothorax (PSP) [MIM:173600]. PSP is a condition in which air is present in the pleural space in the absence of a precipitating event, such as trauma or lung disease. This results in secondary collapse of the lung, either partially or completely, and some degree of hypoxia. PSP is relatively common, with an incidence between 7.4-18/100'000 for men and 1.2-6/100'000 for women and a dose-dependent, increased risk among smokers. Most cases are sporadic, typically occurring in tall, thin men aged 10-30 years and generally while at rest. Familial PSP is rarer and usually is inherited as an autosomal dominant condition with reduced penetrance, although X-linked recessive and autosomal recessive inheritance have also been suggested.
Note=Defects in FLCN may be involved in renal cell carcinoma. -
配列類似性
Belongs to the folliculin family. -
発生段階
Expressed in fetal lung, kidney, liver, and brain. -
翻訳後修飾
Phosphorylated. Several different phosphorylated forms exist. -
細胞内局在
Cytoplasm. Nucleus. Mainly localized in the nucleus. Co-localizes with FNIP1 and FNIP2 in the cytoplasm. - Information by UniProt
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参照データベース
- Entrez Gene: 201163 Human
- Entrez Gene: 216805 Mouse
- Entrez Gene: 303185 Rat
- Omim: 607273 Human
- SwissProt: Q8NFG4 Human
- SwissProt: Q8QZS3 Mouse
- SwissProt: Q76JQ2 Rat
- Unigene: 31652 Human
see all -
別名
- BHD antibody
- BHD skin lesion fibrofolliculoma protein antibody
- Birt Hogg Dube syndrome protein antibody
see all
画像
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All lanes : Anti-FLCN antibody [EPNCIR147] (ab124885) at 1/1000 dilution
Lane 1 : Wild-type HeLa cell lysate
Lane 2 : FLCN knockout HeLa cell lysate
Lane 3 : HeLa cell lysate
Lane 4 : HEK-293 cell lysate
Lysates/proteins at 20 µg per lane.
Performed under reducing conditions.
Predicted band size: 64 kDa
Observed band size: 64 kDaLanes 1 - 4: Merged signal (red and green). Green - ab124885 observed at 64 kDa. Red - loading control ab8245 (Mouse anti-GAPDH antibody [6C5]) observed at 37 kDa.
ab124885 was shown to react with FLCN in wild-type HeLa cells in Western blot with loss of signal observed in FLCN knockout cell line ab265268 (FLCN knockout cell lysate ab257953). Wild-type HeLa and FLCN knockout cell lysates were subjected to SDS-PAGE. Membranes were blocked in fluorescent western blot (TBS-based) blocking solution before incubation with ab124885 and ab8245 (Mouse anti-GAPDH antibody [6C5]) overnight at 4 °C at a 1 in 1000 dilution and a 1 in 20000 dilution respectively. Blots were incubated with Goat anti-Rabbit IgG H&L (IRDye® 800CW) preabsorbed (ab216773) and Goat anti-Mouse IgG H&L (IRDye® 680RD) preabsorbed (ab216776) secondary antibodies at 1 in 20000 dilution for 1 h at room temperature before imaging.
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All lanes : Anti-FLCN antibody [EPNCIR147] (ab124885) at 1/1000 dilution
Lane 1 : 293T cell lysate
Lane 2 : NCCIT cell lysate
Lysates/proteins at 10 µg per lane.
Secondary
All lanes : HRP labelled goat anti-rabbit at 1/2000 dilution
Predicted band size: 64 kDa
Observed band size: 70 kDa why is the actual band size different from the predicted?
データシートおよび資料
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SDS download
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Datasheet download
参考文献 (4)
ab124885 は 4 報の論文で使用されています。
- Li K et al. Folliculin promotes substrate-selective mTORC1 activity by activating RagC to recruit TFE3. PLoS Biol 20:e3001594 (2022). PubMed: 35358174
- Li J et al. Myeloid Folliculin balances mTOR activation to maintain innate immunity homeostasis. JCI Insight 5:N/A (2019). PubMed: 30843872
- Kennedy JC et al. Loss of FLCN inhibits canonical WNT signaling via TFE3. Hum Mol Genet 28:3270-3281 (2019). PubMed: 31272105
- Wada S et al. The tumor suppressor FLCN mediates an alternate mTOR pathway to regulate browning of adipose tissue. Genes Dev 30:2551-2564 (2016). PubMed: 27913603