Sequestosome 1 (SQSTM1) Antibody – Rabbit Polyclonal

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. [provided by RefSeq, Mar 2009]

SQSTM1: an adapter protein which binds ubiquitin, shuttling proteins to the proteasome. Co-localizes along with the E3 ubiquitin ligase, TRAF6, to aggregates from Alzheimer's disease brains but not in control brain. May regulate the activation of NFKB1 by TNF-alpha, nerve growth factor (NGF) and interleukin-1. May play a role in titin downstream signaling in muscle cells. May regulate signaling cascades through ubiquitination. May be involved in cell differentiation, apoptosis, immune response and regulation of K(+) channels. Forms ternary complexes with PKCZ and Kv-beta2 or PKCZ and GABBR3. Also interacts with KCNAB1, GABRR1, GABRR2 and GABRR3. Interacts with EBI3, LCK, RASA1, PKCZ , PKCI, NR2F2, NTRK1, NTRK2, NTRK3, NBR1, MEK5, TRIM55 and MAPKAPK5. Interacts with the proteasome subunits PSMD4 and PSMC2. Interacts with K63- polyubiquitinated MAPT/TAU. Interacts with IKBKB through PRKCZ and PRKCI. Interacts with NGFR through TRAF6 and bridges that complex to NTRK1. Forms a complex with MEK5 and PRKCZ or PRKCI. Component of a ternary complex with PAWR and PRKCZ. Upon TNF-alpha stimulation, interacts with RIPK1 problably bridging IKBKB to the TNF-R1 complex composed of TNF-R1/TNFRSF1A, TRADD and RIPK1. Forms a complex with JUB/Ajuba, PRKCZ and TRAF6. 2 isoforms of the human protein are produced by alternative splicing.

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

Chromosomal Location of Human Ortholog: 5q35.3

Cellular Component: autophagic vacuole; cytoplasm; cytosol; inclusion body; nucleoplasm; PML body

Molecular Function: identical protein binding; protein binding; protein kinase binding; protein kinase C binding; receptor tyrosine kinase binding; SH2 domain binding; ubiquitin binding; ubiquitin protein ligase binding

Biological Process: autophagy; endosome transport; macroautophagy; negative regulation of apoptosis; positive regulation of apoptosis; positive regulation of macroautophagy; positive regulation of transcription from RNA polymerase II promoter; protein localization; regulation of I-kappaB kinase/NF-kappaB cascade; response to stress; 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