Description
Product Usage Information
Western Blotting: 1:1000
Immunoprecipitation: 1:100
Storage
Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/ml BSA, 50% glycerol and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody.
Protocol
Available protocols: Western Blotting, Immunoprecipitation
Specificity / Sensitivity
FLCN (D14G9) Rabbit Monoclonal Antibody detects endogenous levels of total FLCN protein.
Species Reactivity: Human
Source / Purification
Monoclonal antibody is produced by immunizing animals with recombinant human FLCN protein.
Background
The protein folliculin (FLCN) is encoded by the BHD (Birt-Hogg-Dube) gene that is altered in BHD Syndrome, a disorder characterized by the presence of benign connective tissue tumors known as fibrofolliculomas, renal tumors and lung cysts (1). Clinical similarities between BHD and hamartoma-producing disorders caused by Tsc2, PTEN and LKB1 gene mutations indicate that FLCN might also be important in nutrient and energy sensing through the mTOR pathway (2). This model is supported by studies demonstrating a direct correlation between the down regulation of BHD and a reduction in mTOR associated phosphorylation of S6 ribosomal protein (3). Mutation of either the TSC1 or TSC2 gene results in elevated mTOR activity (4) while deletion of the Tsc2 and BHD homologs in yeast have opposing effects on both mTOR signaling and amino acid homeostasis (5). BHD knock-out mice develop cysts and renal cell tumors similar to those found in BHD patients along with low levels of phosphorylated S6 ribosomal protein (3). Based on these finding, it appears that either abnormally high or abnormally low levels of mTOR signaling might contribute to renal cell carcinogenesis.
Alternate Names
BHD; BHD skin lesion fibrofolliculoma protein; Birt-Hogg-Dube syndrome protein; DENND8B; DKFZp547A118; FLCL; FLCN; FLJ45004; FLJ99377; Folliculin; MGC17998; MGC23445
