FLCN, Blocking Peptide

Folliculin; BHD skin lesion fibrofolliculoma protein; Birt-Hogg-Dube syndrome protein; FLCN; BHD

For Research Use Only. Not for use in diagnostic procedures.

The synthetic peptide sequence used to generate the antibody was selected from the Center region of human FLCN. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.

Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Small volumes of FLCN blocking peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

20,877 Da

folliculin

folliculin

Folliculin

BHD  [Similar Products]

FLCN_HUMAN

This gene is located within the Smith-Magenis syndrome region on chromosome 17. Mutations in this gene are associated with Birt-Hogg-Dube syndrome, which is characterized by fibrofolliculomas, renal tumors, lung cysts, and pneumothorax. Alternative splicing of this gene results in two transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008]

FLCN: a protein that is expressed in most tissues and that has no significant homology to other proteins. Involved in nutrient sensing through the AMPK and mTOR signaling pathways. Regulates lysosome function by promoting the mTORC1-dependent sequestration of transcription factor EB (TFEB). Required for the recruitment of mTORC1 to lysosomes by Rag GTPases. Amino acid depletion leads to its recruitment to the surface of lysosomes where it directly binds RagA, an interaction that is promoted by FNIP1. May be a tumor suppressor. Loss of function mutations cause Birt-Hogg-Dube syndrome (BHD), a disease characterized by fibrofolliculomas (hair follicle tumors), and predispositions for renal carcinoma, pneumothorax, and pulmonary cysts. These symptoms are similar to those that occur in TSC or tuberous sclerosis complex, suggesting the involvement in the same pathways as TSC1. Belongs to the folliculin family. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Tumor suppressor

Chromosomal Location of Human Ortholog: 17p11.2

Cellular Component: cytoplasm; lysosome; midbody; nucleus; plasma membrane

Molecular Function: protein binding; protein complex binding

Biological Process: hemopoiesis; in utero embryonic development; intercellular junction assembly; negative regulation of cell growth; negative regulation of cell migration; negative regulation of protein kinase B signaling cascade; negative regulation of Rho protein signal transduction; negative regulation of TOR signaling pathway; negative regulation of transcription from RNA polymerase II promoter; positive regulation of apoptosis; positive regulation of autophagy; positive regulation of cell adhesion; positive regulation of protein amino acid phosphorylation; positive regulation of TOR signaling pathway; positive regulation of transcription from RNA polymerase II promoter; positive regulation of transforming growth factor beta receptor signaling pathway; regulation of cytokinesis; regulation of histone acetylation; regulation of protein amino acid phosphorylation; regulation of TOR signaling pathway; TOR signaling pathway

Disease: Birt-hogg-dube Syndrome; Colorectal Cancer; Pneumothorax, Primary Spontaneous; Renal Cell Carcinoma, Nonpapillary

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