|Other Names||Sequestosome-1, EBI3-associated protein of 60 kDa, EBIAP, p60, Phosphotyrosine-independent ligand for the Lck SH2 domain of 62 kDa, Ubiquitin-binding protein p62, SQSTM1, ORCA, OSIL|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Autophagy receptor that interacts directly with both the cargo to become degraded and an autophagy modifier of the MAP1 LC3 family. Required both for the formation and autophagic degradation of polyubiquitin-containing bodies, called ALIS (aggresome-like induced structures) and links ALIS to the autophagic machinery. Involved in midbody ring degradation. May regulate the activation of NFKB1 by TNF-alpha, nerve growth factor (NGF) and interleukin- 1. May play a role in titin/TTN downstream signaling in muscle cells. May regulate signaling cascades through ubiquitination. Adapter that mediates the interaction between TRAF6 and CYLD (By similarity). May be involved in cell differentiation, apoptosis, immune response and regulation of K(+) channels.|
|Cellular Location||Cytoplasm. Late endosome. Lysosome. Cytoplasmic vesicle, autophagosome. Nucleus. Endoplasmic reticulum. Cytoplasm, P-body. Note=Sarcomere (By similarity). In cardiac muscles localizes to the sarcomeric band (By similarity) Commonly found in inclusion bodies containing polyubiquitinated protein aggregates. In neurodegenerative diseases, detected in Lewy bodies in Parkinson disease, neurofibrillary tangles in Alzheimer disease, and HTT aggregates in Huntington disease. In protein aggregate diseases of the liver, found in large amounts in Mallory bodies of alcoholic and nonalcoholic steatohepatitis, hyaline bodies in hepatocellular carcinoma, and in SERPINA1 aggregates. Enriched in Rosenthal fibers of pilocytic astrocytoma In the cytoplasm, observed in both membrane-free ubiquitin- containing protein aggregates (sequestosomes) and membrane- surrounded autophagosomes. Colocalizes with TRIM13 in the perinuclear endoplasmic reticulum. Co-localizes with TRIM5 in the cytoplasmic bodies.|
|Tissue Location||Ubiquitously expressed.|
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This gene encodes a multifunctional protein that bindsubiquitin and regulates activation of the nuclear factor kappa-B(NF-kB) signaling pathway. The protein functions as ascaffolding/adaptor protein in concert with TNF receptor-associatedfactor 6 to mediate activation of NF-kB in response to upstreamsignals. Alternatively spliced transcript variants encoding eitherthe same or different isoforms have been identified for this gene.Mutations in this gene result in sporadic and familial Pagetdisease of bone.
Visconti, M.R., et al. J. Bone Miner. Res. 25(11):2368-2373(2010)Ding, W.X., et al. J. Biol. Chem. 285(36):27879-27890(2010)Gao, C., et al. Nat. Cell Biol. 12(8):781-790(2010)Jain, A., et al. J. Biol. Chem. 285(29):22576-22591(2010)Lau, A., et al. Mol. Cell. Biol. 30(13):3275-3285(2010)
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