SQSTM1 Autophagy receptor required for selective macroautophagy (aggrephagy). Functions as a bridge between polyubiquitinated cargo and autophagosomes. Interacts directly with both the cargo to become degraded and an autophagy modifier of the MAP1 LC3 family. Along with WDFY3, involved in the formation and autophagic degradation of cytoplasmic ubiquitin-containing inclusions (p62 bodies, ALIS/aggresome-like induced structures). Along with WDFY3, required to recruit ubiquitinated proteins to PML bodies in the nucleus. 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. May be involved in cell differentiation, apoptosis, immune response and regulation of K(+) channels. Involved in endosome organization by retaining vesicles in the perinuclear cloud: following ubiquitination by RNF26, attracts specific vesicle-associated adapters, forming a molecular bridge that restrains cognate vesicles in the perinuclear region and organizes the endosomal pathway for efficient cargo transport. Promotes relocalization of 'Lys-63'-linked ubiquitinated TMEM173/STING to autophagosomes. Ubiquitously expressed. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.

Protein type: Autophagy; Nuclear receptor co-regulator; Ubiquitin conjugating system

Chromosomal Location of human Ortholog: 5q35.3

Cellular Component: aggresome; amphisome; autolysosome; autophagosome; cytoplasm; cytosol; endoplasmic reticulum; inclusion body; intracellular membrane-bounded organelle; late endosome; Lewy body; mitochondrion; nucleoplasm; P-body; phagophore assembly site; PML body; sarcomere; sperm midpiece

Molecular Function: enzyme binding; identical protein binding; ionotropic glutamate receptor binding; K63-linked polyubiquitin modification-dependent protein binding; protein binding; protein kinase binding; protein kinase C binding; protein serine/threonine kinase activity; protein-containing complex binding; protein-macromolecule adaptor activity; receptor tyrosine kinase binding; SH2 domain binding; signaling adaptor activity; signaling receptor activity; ubiquitin binding; ubiquitin protein ligase binding; ubiquitin-dependent protein binding; zinc ion binding

Biological Process: aggrephagy; apoptotic process; autophagy; autophagy of mitochondrion; brown fat cell proliferation; cell differentiation; cellular response to reactive oxygen species; endosomal transport; endosome organization; energy homeostasis; immune system process; intracellular signal transduction; macroautophagy; mitophagy; negative regulation of protein ubiquitination; negative regulation of transcription by RNA polymerase II; pexophagy; positive regulation of apoptotic process; positive regulation of autophagy; positive regulation of long-term synaptic potentiation; positive regulation of protein localization to plasma membrane; positive regulation of protein phosphorylation; positive regulation of transcription by RNA polymerase II; protein catabolic process; protein import into nucleus; protein localization; protein localization to perinuclear region of cytoplasm; protein phosphorylation; protein targeting to vacuole involved in autophagy; regulation of I-kappaB kinase/NF-kappaB signaling; regulation of mitochondrion organization; regulation of protein complex stability; regulation of Ras protein signal transduction; response to ischemia; response to mitochondrial depolarisation; selective autophagy; temperature homeostasis; transcription by RNA polymerase II; ubiquitin-dependent protein catabolic process

Disease: Frontotemporal Dementia And/or Amyotrophic Lateral Sclerosis 3; Myopathy, Distal, With Rimmed Vacuoles; Neurodegeneration With Ataxia, Dystonia, And Gaze Palsy, Childhood-onset; Paget Disease Of Bone 3

Reference #: Q13501 (UniProtKB)

Alt. Names/Synonyms: A170; autophagy receptor p62; DMRV; EBI3-associated protein of 60 kDa; EBI3-associated protein p60; EBIAP; FTDALS3; NADGP; ORCA; OSIL; oxidative stress induced like; p60; p62; p62B; PDB3; phosphotyrosine independent ligand for the Lck SH2 domain p62; Phosphotyrosine-independent ligand for the Lck SH2 domain of 62 kDa; sequestosome 1; Sequestosome-1; SQSTM; SQSTM1; Ubiquitin-binding protein p62; ZIP3

Gene Symbols: SQSTM1

Molecular weight: 47,687 Da

Basal Isoelectric point: 5.1 Predict pI for various phosphorylation states

Protein-Specific Antibodies, siRNAs or Recombinant Proteins from Cell Signaling Technology^®

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SQSTM1

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