- Catalog number
- A00662-2
- Size / count
- 100 µg/Vial
- Dry ice
- No
- Hazardous
- No
- Discontinued
- No
- Background
- Transcription factor EB is a protein that in humans is encoded by the TFEB gene. TFEB is a master gene for lysosomal biogenesis. It encodes a transcription factor that coordinates expression of lysosomal hydrolases, membrane proteins and genes involved in autophagy. Upon nutrient depletion and under aberrant lysosomal storage conditions such as in lysosomal storage diseases, TFEB translocates from the cytoplasm to the nucleus, resulting in the activation of its target genes. TFEB overexpression in cultured cells induces lysosomal biogenesis, exocytosis and autophagy. Viral-mediated TFEB overexpression in cellular and mouse models of lysosomal storage disorders and in common neurodegenerative diseases such as Huntington, Parkinson and Alzheimer diseases, resulted in intracellular clearance of accumulating molecules and rescue of disease phenotypes. TFEB is activated by PGC1-alpha and promotes reduction of htt aggregation and neurotoxicity in a mouse model of Huntington disease. TFEB overexpression has been found in patients with renal cell carcinoma and pancreatic cancer and was shown to promote tumorogenesis via induction of varius oncogenic signals.
- Synonyms
- NKG2-D type II integral membrane protein; Killer cell lectin-like receptor subfamily K member 1; NK cell receptor D; NKG2-D-activating NK receptor; CD314; KLRK1; D12S2489E; NKG2D
- Gene Name
- TFEB
- Gene ID
- 7942
- UniProt
- P19484
- Host
- Rabbit
- Reactivity
- Human, Mouse, Rat
- Cross Reactivity
- No cross-reactivity with other proteins.
- Immunogen
- E.coli-derived human TFEB recombinant protein (Position: E68-L350) .
- Clonality
- Polyclonal
- Tissue Specificity
- Expressed in natural killer (NK) cells, CD8 (+) alpha-beta and gamma-delta T-cells. Expressed on essentially all CD56+CD3- NK cells from freshly isolated PBMC. Expressed in interferon-producing killer dendritic cells (IKDCs) .
- Applications
- WB, ELISA
- Field of Research
- Immunoglobulins, Immunology, Innate Immunity, Macrophage/Inflammation, Receptors
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
- Function
- Function as an activating and costimulatory receptor involved in immunosurveillance upon binding to various cellular stress-inducible ligands displayed at the surface of autologous tumor cells and virus-infected cells. Provides both stimulatory and costimulatory innate immune responses on activated killer (NK) cells, leading to cytotoxic activity. Acts as a costimulatory receptor for T-cell receptor (TCR) in CD8 (+) T-cell-mediated adaptive immune responses by amplifying T-cell activation. Stimulates perforin-mediated elimination of ligand-expressing tumor cells. Signaling involves calcium influx, culminating in the expression of TNF-alpha. Participates in NK cell-mediated bone marrow graft rejection. May play a regulatory role in differentiation and survival of NK cells. Binds to ligands belonging to various subfamilies of MHC class I-related glycoproteins including MICA, MICB, RAET1E, RAET1G, ULBP1, ULBP2, ULBP3 (ULBP2>ULBP1>ULBP3) and ULBP4.
- References & Citations
- 1. Argani, P., Hawkins, A., Griffin, C. A., Goldstein, J. D., Haas, M., Beckwith, J. B., Mankinen, C. B., Perlman, E. J. A distinctive pediatric renal neoplasm characterized by epithelioid morphology, basement membrane production, focal HMB45 immunoreactivity, and t (6;11) (p21.1; q12) chromosome translocation. Am. J. Path. 158: 2089-2096, 2001. 2. Carr, C. S., Sharp, P. A. A helix-loop-helix protein related to the immunoglobulin E box-binding proteins. Molec. Cell. Biol. 10: 4384-4388, 1990. 3. Davis, I. J., Hsi, B.-L., Arroyo, J. D., Vargas, S. O., Yeh, Y. A., Motyckova, G., Valencia, P., Perez-Atayde, A. R., Argani, P., Ladanyi, M., Fletcher, J. A., Fisher, D. E. Cloning of an alpha-TFEB fusion in renal tumors harboring the t (6;11) (p21; q13) chromosome translocation. Proc. Nat. Acad. Sci. 100: 6051-6056, 2003.
- Storage Conditions
- Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
- Calculated Molecular Weight
- 53 kDa
- Observed Molecular Weight
- 65 kDa
- Gene Name Synonym
- Killer cell lectin like receptor K1
- Subcellular Location
- Cell membrane.
- Isotype
- Rabbit IgG
- Contents
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl and 0.2 mg Na2HPO4.
