Recombinant Human Sequestosome-1 (SQSTM1)

Uniprot Id

Q13501

Target Species

Human

Target Name

SQSTM1

Target Full Name

Sequestosome-1

Target Function

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 STING1 to autophagosomes. Acts as an activator of the NFE2L2/NRF2 pathway via interaction with KEAP1: interaction inactivates the BCR(KEAP1) complex, promoting nuclear accumulation of NFE2L2/NRF2 and subsequent expression of cytoprotective genes.

Target Involvement

Paget disease of bone 3 (PDB3); Frontotemporal dementia and/or amyotrophic lateral sclerosis 3 (FTDALS3); Neurodegeneration with ataxia, dystonia, and gaze palsy, childhood-onset (NADGP); Myopathy, distal, with rimmed vacuoles (DMRV)

Target Subcellular Location

Cytoplasm, cytosol. Late endosome. Lysosome. Cytoplasmic vesicle, autophagosome. Nucleus. Endoplasmic reticulum. Nucleus, PML body. Cytoplasm, myofibril, sarcomere.

Target Tissue Specificity

Ubiquitously expressed.

Target Synonyms

A170; DMRV; EBI 3 associated protein of 60 kDa; EBI 3 associated protein p60; EBI3 associated protein of 60 kDa; EBI3 associated protein p60; EBI3-associated protein of 60 kDa; EBIAP; FTDALS3; MGC127197; ORCA; OSF-6; Osi; OSIL; Oxidative stress induced like; p60; p62; p62B; Paget disease of bone 3; PDB 3; PDB3; Phosphotyrosine independent ligand for the Lck SH2 domain of 62 kDa; Phosphotyrosine independent ligand for the Lck SH2 domain p62; Phosphotyrosine-independent ligand for the Lck SH2 domain of 62 kDa; PKC-zeta-interacting protein; Protein kinase C-zeta-interacting protein; Sequestosome 1; Sequestosome-1; SQSTM 1; SQSTM_HUMAN; Sqstm1; STAP; STONE14; Ubiquitin binding protein p62; Ubiquitin-binding protein p62; ZIP 3; ZIP; ZIP3

Target Background

This gene encodes a multifunctional protein that binds ubiquitin and regulates activation of the nuclear factor kappa-B (NF-kB) signaling pathway. The protein functions as a scaffolding/adaptor protein in concert with TNF receptor-associated factor 6 to mediate activation of NF-kB in response to upstream signals. Alternatively spliced transcript variants encoding either the same or different isoforms have been identified for this gene. Mutations in this gene result in sporadic and familial Paget disease of bone.