Protein: Q13501

UniprotKB AC UniprotKB ID Gene name Full name Species Curated set
Q13501 (Uniprot) SQSTM_HUMAN SQSTM1 Sequestosome-1 human No
Uniprot: Autophagy receptor that interacts directly with both the cargo to become degraded and an autophagy modifier of the MAP1 LC3 family (PubMed:16286508, PubMed:20168092, PubMed:24128730, PubMed:28404643). Along with WDFY3, involved in the formation and autophagic degradation of cytoplasmic ubiquitin-containing inclusions (p62 bodies, ALIS/aggresome-like induced structures). Along with SQSTM1, required to recruit ubiquitinated proteins to PML bodies in the nucleus (PubMed:24128730, PubMed:20168092). 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. 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 (PubMed:27368102). more..
MoonDB Dissimilar Functions
GO ID 1 Function 1 Module ID 1 GO ID 2 Function 2 Module ID 2 Association Probability (PrOnto) Interaction Probability (PrOnto)
GO:0043170 macromolecule metabolic process 310 GO:0033036 macromolecule localization 517 2.48e-08 1.95e-27
GO:0033036 macromolecule localization 517 GO:0006725 cellular aromatic compound metabolic process 54, 585 1.29e-20 6.46e-74
Biological Processes
Module ID (MoonGO) GO ID (BP) GO Name
54 GO:0010467 gene expression
54 GO:0006139 nucleobase-containing compound metabolic process
54 GO:0060255 regulation of macromolecule metabolic process
134 GO:0044267 cellular protein metabolic process
134 GO:0060255 regulation of macromolecule metabolic process
134 GO:0007165 signal transduction
248 GO:0044267 cellular protein metabolic process
248 GO:0045893 positive regulation of transcription, DNA-templated
248 GO:0051246 regulation of protein metabolic process
248 GO:0007165 signal transduction
310 GO:0019538 protein metabolic process
404 GO:0044267 cellular protein metabolic process
404 GO:0007165 signal transduction
517 GO:0008104 protein localization
517 GO:0019538 protein metabolic process
585 GO:0006139 nucleobase-containing compound metabolic process
Cellular Components
Module ID (MoonGO) GO ID (CC) GO Name
30 GO:0005829 cytosol
30 GO:0043232 intracellular non-membrane-bounded organelle
54 GO:0070013 intracellular organelle lumen
54 GO:0005634 nucleus
134 GO:0005829 cytosol
134 GO:0005634 nucleus
248 GO:0031981 nuclear lumen
310 GO:0005829 cytosol
310 GO:0005634 nucleus
404 GO:0005829 cytosol
404 GO:0012505 endomembrane system
404 GO:0031982 vesicle
517 GO:0005829 cytosol
517 GO:0012505 endomembrane system
517 GO:0031982 vesicle
585 GO:0031981 nuclear lumen
GO ID (BP) GO Name Evidence Code (GO EC)
GO:0000122 negative regulation of transcription from RNA polymerase II promoter IEA
GO:0000422 autophagy of mitochondrion NAS
GO:0000423 mitophagy IGI
GO:0001934 positive regulation of protein phosphorylation IEA
GO:0002376 immune system process IEA
GO:0002931 response to ischemia IEA
GO:0006468 protein phosphorylation IEA
GO:0006511 ubiquitin-dependent protein catabolic process TAS
GO:0006914 autophagy TAS
GO:0006915 apoptotic process IEA
GO:0006950 response to stress TAS
GO:0007032 endosome organization IDA
GO:0008104 protein localization TAS
GO:0010821 regulation of mitochondrion organization NAS
GO:0016197 endosomal transport TAS
GO:0016236 macroautophagy TAS
GO:0030154 cell differentiation IEA
GO:0035556 intracellular signal transduction TAS
GO:0035973 aggrephagy IPI
GO:0043065 positive regulation of apoptotic process TAS
GO:0043066 negative regulation of apoptotic process TAS
GO:0043122 regulation of I-kappaB kinase/NF-kappaB signaling IMP
GO:0044130 negative regulation of growth of symbiont in host IEA
GO:0045944 positive regulation of transcription from RNA polymerase II promoter TAS
GO:0046578 regulation of Ras protein signal transduction NAS
GO:0051291 protein heterooligomerization IEA
GO:0061635 regulation of protein complex stability IDA
GO:0098780 response to mitochondrial depolarisation IGI
GO:1900273 positive regulation of long-term synaptic potentiation ISS
GO:1903078 positive regulation of protein localization to plasma membrane ISS
GO:1905719 protein localization to perinuclear region of cytoplasm IDA
GO ID (CC) GO Name Evidence Code (GO EC)
GO:0000407 pre-autophagosomal structure IEA
GO:0000932 P-body IDA
GO:0005654 nucleoplasm TAS
GO:0005737 cytoplasm IDA
GO:0005770 late endosome IEA
GO:0005776 autophagosome IDA
GO:0005783 endoplasmic reticulum IEA
GO:0005829 cytosol TAS
GO:0016234 inclusion body IDA
GO:0016235 aggresome IBA
GO:0016605 PML body IDA
GO:0043231 intracellular membrane-bounded organelle IDA
GO:0044753 amphisome IDA
GO:0044754 autolysosome IDA
GO:0070062 extracellular exosome IDA
GO:0097225 sperm midpiece IEA
GO:0097413 Lewy body IEA
GO ID 1 Component 1 GO ID 2 Component 2 Association Probability (PrOnto) Interaction Probability (PrOnto)
GO:0005654 nucleoplasm GO:0005783 endoplasmic reticulum 1.84e-53 1.21e-118
GO:0005654 nucleoplasm GO:0070062 extracellular exosome 3.37e-20 1.23e-35
PMID Article Title
8551575 A novel interleukin-12 p40-related protein induced by latent Epstein-Barr virus infection in B lymphocytes.
8618896 Phosphotyrosine-independent binding of a 62-kDa protein to the src homology 2 (SH2) domain of p56lck and its regulation by phosphorylation of Ser-59 in the lck unique N-terminal region.
8650207 Molecular cloning of a phosphotyrosine-independent ligand of the p56lck SH2 domain.
8702753 p62, a phosphotyrosine-independent ligand of the SH2 domain of p56lck, belongs to a new class of ubiquitin-binding proteins.
8910285 A p56(lck) ligand serves as a coactivator of an orphan nuclear hormone receptor.
9566925 Localization of atypical protein kinase C isoforms into lysosome-targeted endosomes through interaction with p62.
9762895 Genomic structure and promoter analysis of the p62 gene encoding a non-proteasomal multiubiquitin chain binding protein.
10356400 The interaction of p62 with RIP links the atypical PKCs to NF-kappaB activation.
10362795 Analysis of intracytoplasmic hyaline bodies in a hepatocellular carcinoma. Demonstration of p62 as major constituent.
10708586 p62 functions as a p38 MAP kinase regulator.
10747026 The atypical PKC-interacting protein p62 channels NF-kappaB activation by the IL-1-TRAF6 pathway.
11244088 The atypical protein kinase C-interacting protein p62 is a scaffold for NF-kappaB activation by nerve growth factor.
11755531 p62 forms a ternary complex with PKCzeta and PAR-4 and antagonizes PAR-4-induced PKCzeta inhibition.
11786419 p62 Is a common component of cytoplasmic inclusions in protein aggregation diseases.
11981755 Mallory body -- a disease-associated type of sequestosome.
11992264 Recurrent mutation of the gene encoding sequestosome 1 (SQSTM1/p62) in Paget disease of bone.
12374763 Domain-specific mutations in sequestosome 1 (SQSTM1) cause familial and sporadic Paget's disease.
12471037 Association of the atypical protein kinase C-interacting protein p62/ZIP with nerve growth factor receptor TrkA regulates receptor trafficking and Erk5 signaling.
12700667 p62 overexpression in breast tumors and regulation by prostate-derived Ets factor in breast cancer cells.
12813044 Interaction codes within the family of mammalian Phox and Bem1p domain-containing proteins.
12857745 Structure of the ubiquitin-associated domain of p62 (SQSTM1) and implications for mutations that cause Paget's disease of bone.
12887891 PB1 domain-mediated heterodimerization in NADPH oxidase and signaling complexes of atypical protein kinase C with Par6 and p62.
14584883 Three novel mutations in SQSTM1 identified in familial Paget's disease of bone.
14702039 Complete sequencing and characterization of 21,243 full-length human cDNAs.
15125799 Two novel mutations at exon 8 of the Sequestosome 1 (SQSTM1) gene in an Italian series of patients affected by Paget's disease of bone (PDB).
15146436 Familial Paget's disease in The Netherlands: occurrence, identification of new mutations in the sequestosome 1 gene, and their clinical associations.
15158159 Transcriptional activation of p62/A170/ZIP during the formation of the aggregates: possible mechanisms and the role in Lewy body formation in Parkinson's disease.
15176995 Novel UBA domain mutations of SQSTM1 in Paget's disease of bone: genotype phenotype correlation, functional analysis, and structural consequences.
15207768 Identification of SQSTM1 mutations in familial Paget's disease in Australian pedigrees.
15340068 Sequestosome 1/p62 is a polyubiquitin chain binding protein involved in ubiquitin proteasome degradation.
15372022 The DNA sequence and comparative analysis of human chromosome 5.
15489334 The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
15592455 Immunoaffinity profiling of tyrosine phosphorylation in cancer cells.
15802564 The kinase domain of titin controls muscle gene expression and protein turnover.
15870274 The LIM protein Ajuba influences interleukin-1-induced NF-kappaB activation by affecting the assembly and activity of the protein kinase Czeta/p62/TRAF6 signaling complex.
15911346 Inhibition of sequestosome 1/p62 up-regulation prevents aggregation of ubiquitinated proteins induced by prostaglandin J2 without reducing its neurotoxicity.
15953362 Sequestosome 1/p62 shuttles polyubiquitinated tau for proteasomal degradation.
16079148 The p62 scaffold regulates nerve growth factor-induced NF-kappaB activation by influencing TRAF6 polyubiquitination.
16286508 p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death.
16964243 A probability-based approach for high-throughput protein phosphorylation analysis and site localization.
17081983 Global, in vivo, and site-specific phosphorylation dynamics in signaling networks.
17488105 Detection and validation of non-synonymous coding SNPs from orthogonal analysis of shotgun proteomics data.
17580304 p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy.
17932931 Conformation and dynamics of the three-helix bundle UBA domain of p62 from experiment and simulation.
18083707 Ubiquitin recognition by the ubiquitin-associated domain of p62 involves a novel conformational switch.
18669648 A quantitative atlas of mitotic phosphorylation.
18691976 Kinase-selective enrichment enables quantitative phosphoproteomics of the kinome across the cell cycle.
19369195 Large-scale proteomics analysis of the human kinome.
19413330 Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach.
19690332 Quantitative phosphoproteomic analysis of T cell receptor signaling reveals system-wide modulation of protein-protein interactions.
19931284 Dimerisation of the UBA domain of p62 inhibits ubiquitin binding and regulates NF-kappaB signalling.
20068231 Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis.
20168092 p62/SQSTM1 and ALFY interact to facilitate the formation of p62 bodies/ALIS and their degradation by autophagy.
20357094 p62/sequestosome-1 associates with and sustains the expression of retroviral restriction factor TRIM5alpha.
21149568 Formin follows function: a muscle-specific isoform of FHOD3 is regulated by CK2 phosphorylation and promotes myofibril maintenance.
21269460 Initial characterization of the human central proteome.
21406692 System-wide temporal characterization of the proteome and phosphoproteome of human embryonic stem cell differentiation.
21923101 Independent interactions of ubiquitin-binding domains in a ubiquitin-mediated ternary complex.
22084127 SQSTM1 mutations in familial and sporadic amyotrophic lateral sclerosis.
22178386 TRIM13 regulates ER stress induced autophagy and clonogenic ability of the cells.
22421968 TP53INP1, a tumor suppressor, interacts with LC3 and ATG8-family proteins through the LC3-interacting region (LIR) and promotes autophagy-dependent cell death.
22814378 N-terminal acetylome analyses and functional insights of the N-terminal acetyltransferase NatB.
23186163 Toward a comprehensive characterization of a human cancer cell phosphoproteome.
23274085 Sestrins activate Nrf2 by promoting p62-dependent autophagic degradation of Keap1 and prevent oxidative liver damage.
23908376 The LIR motif - crucial for selective autophagy.
24042580 SQSTM1 mutations in French patients with frontotemporal dementia or frontotemporal dementia with amyotrophic lateral sclerosis.
24089205 Autophagy promotes primary ciliogenesis by removing OFD1 from centriolar satellites.
24128730 TRAF6 mediates ubiquitination of KIF23/MKLP1 and is required for midbody ring degradation by selective autophagy.
24275569 An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.
24668264 Structural determinants in GABARAP required for the selective binding and recruitment of ALFY to LC3B-positive structures.
24899140 Rare mutations in SQSTM1 modify susceptibility to frontotemporal lobar degeneration.
25040165 Sestrin2 promotes Unc-51-like kinase 1 mediated phosphorylation of p62/sequestosome-1.
25114083 A phenotype of atypical apraxia of speech in a family carrying SQSTM1 mutation.
25127057 TRIM proteins regulate autophagy and can target autophagic substrates by direct recognition.
25422469 Disruption of FAT10-MAD2 binding inhibits tumor progression.
26208961 SQSTM1 splice site mutation in distal myopathy with rimmed vacuoles.
27368102 An ER-associated pathway defines endosomal architecture for controlled cargo transport.
27545679 Absence of the autophagy adaptor SQSTM1/p62 causes childhood-onset neurodegeneration with ataxia, dystonia, and gaze palsy.
28112733 Site-specific mapping of the human SUMO proteome reveals co-modification with phosphorylation.
28404643 The BEACH-containing protein WDR81 coordinates p62 and LC3C to promote aggrephagy.
OMIM ID Disease Name
167250 Paget disease of bone 3
616437 Frontotemporal dementia and/or amyotrophic lateral sclerosis 3
617145 Neurodegeneration with ataxia, dystonia, and gaze palsy, childhood-onset
617158 Myopathy, distal, with rimmed vacuoles
Domain Name Domain ID Source
PB1_dom IPR000270 InterPro
Znf_ZZ IPR000433 InterPro
UBA-like_sf IPR009060 InterPro
UBA IPR015940 InterPro
SQSTM_UBA IPR033741 InterPro
PB1_p62 IPR034866 InterPro
PB1 PF00564 Pfam
ZZ PF00569 Pfam
UBA_5 PF16577 Pfam
ZF_ZZ_1 PS01357 PROSITE
UBA PS50030 PROSITE
ZF_ZZ_2 PS50135 PROSITE
PB1 PS51745 PROSITE
UBA SM00165 SMART
ZnF_ZZ SM00291 SMART
PB1 SM00666 SMART
SSF46934 SSF46934 SUPFAM
PB1_p62 cd06402 CDD
UBA_SQSTM cd14320 CDD