FNIP1 (Folliculin interacting protein 1, DKFZp686E18167, DKFZp781P0215, KIAA1961, MGC667)

The FNIP1 gene consists of a 6655 nt mRNA (using NM 133372 derived from AC005593.1, DQ145719.1, AC008695.9 and AL832008.1, the coding sequence extends from nt143 to nt3643) and contains 18 coding exons. The initiation codon is located within exon 1. Northern blot analysis revealed an about 7 kb FNIP1 mRNA transcript that was expressed in most major adult tissues, with strongest expression in heart, liver and placenta, and expression in kidney and lung, tissues involved in the Birt-Hogg-Dube' syndrome phenotype (see below). Several alternate transcripts lacking one or more exons have been reported. Transcript 1 is the full-length isoform. Transcript 2 lacks exon 7 (NM 001008738). The FNIP1 protein contains 1166 amino acids and has an estimated molecular weight of 130kD. Epitope-tagged FNIP1 expressed in HeLa cells localized exclusively in the cytoplasm. Coimmunoprecipitation studies to elucidate the function of folliculin (FLCN) (encoded by the tumor suppressor gene, BHD/FLCN, which is mutated in the Birt-Hogg-Dube' syndrome) identified a novel folliculin-interacting protein, FNIP1, which interacts through the C-terminus of FLCN. FNIP1 overexpression enhanced phosphorylation of FLCN and phospho-FLCN preferentially bound to FNIP1.\n\nFNIP1 is a novel protein with no domains to suggest function. By coimmunoprecipitation studies FNIP1 was also found to interact with the heterotrimer, 5'AMP-activated protein kinase (AMPK), a key molecule for energy sensing and a negative regulator of mTOR (mammalian target of rapamycin). AMPK, which bound to FNIP1, was preferentially in its phosphorylated (active) form and FNIP1 could act as a substrate for AMPK phosphorylation both in vitro and in vivo. Inhibition of AMPK kinase activity resulted in reduced FNIP1 protein expression in HEK293 cells suggesting that phosphorylation of FNIP1 by AMPK may enhance protein stability. These data suggest that FNIP1 and its interacting partner, FLCN, may be involved in energy and nutrient-sensing through the AMPK and mTOR signaling pathways. FNIP1 was also shown to interact with HSP90 by coimmunoprecipitation in HEK293 cells. A comparison of FNIP1 proteins across species identified 5 blocks of conserved sequence with at least 35% similarity. FNIP1 homologs have been identified in Mus musculus, Gallus gallus, Xenopus tropicalis, Danio rerio, Drosophila melanogaster and Caenorhabditis elegans. FNIP1 interacts with FLCN, encoded by a novel tumor suppressor gene, BHD/FLCN, which is mutated in the germline of patients with BHD syndrome. Genodermatosis characterized by the triad of benign tumors of the hair follicle, spontaneous pneumothorax and kidney tumors.\n\nApplications: \nSuitable for use in ELISA. Other applications not tested.\n\nRecommended Dilution:\nPeptide ELISA: 1:32,000. \nWestern Blot: Preliminary experiments in Human Heart, Liver, Ovary and Spleen lysates gave no specific signal but low background (at antibody concentration up to 1ug/ml). Optimal dilutions to be determined by the researcher.\n\nStorage and Stability: \nMay be stored at 4 degrees C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and store at -20 degrees C. Aliquots are stable for at least 12 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.\n\nPeptide Blocking:\nCorresponding peptide is available for Peptide Blocking studies. See F6002-66-P. Antibody

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SPECIFICATIONS

Catalog Number

F6002-66

Size

100ug

Applications

ELISA

Hosts

Goat

Reactivities

Hum

Form

Supplied as a liquid in Tris saline, pH 7.2, 0.5% BSA, 0.02% sodium azide.

P Type

Pab

Purity

Purified by affinity chromatography.

References

1. Folliculin encoded by the BHD gene interacts with a binding protein, FNIP1, and AMPK, and is involved in AMPK and mTOR signaling., Baba M, Hong SB, Sharma N, Warren MB, Nickerson ML, Iwamatsu A, Esposito D, Gillette WK, Hopkins RF 3rd, Hartley JL, Furihata M, Oishi S, Zhen W, Burke TR Jr, Linehan WM, Schmidt LS, Zbar B., Proc Natl Acad Sci U S A. 2006 Oct 17;103(42):15552-7. Epub 2006 Oct 6.\n2. Atlas of Genetics and Cytogenetics in Oncology and Haematology

Additional Info

Recognizes human FNIP1.

Alternative Names

FNIP1; folliculin interacting protein 1; DKFZp686E18167; DKFZp781P0215; KIAA1961; MGC667; folliculin-interacting protein 1