SQSTM1 Antibody (Center S207) Blocking Peptide
- Known as:
- SQSTM1 Antibody (Center S207) Blocking Peptide
- Catalog number:
- BP19360c
- Product Quantity:
- 2
- Category:
- -
- Supplier:
- Abgen
- Gene target:
- SQSTM1 Antibody (Center S207) Blocking Peptide
Related genes to: SQSTM1 Antibody (Center S207) Blocking Peptide
- Gene:
- RNA5SP207 NIH gene
- Name:
- RNA, 5S ribosomal pseudogene 207
- Previous symbol:
- RN5S207
- Synonyms:
- -
- Chromosome:
- 6p21.1
- Locus Type:
- pseudogene
- Date approved:
- 2011-11-04
- Date modifiied:
- 2014-11-19
- Gene:
- SQSTM1 NIH gene
- Name:
- sequestosome 1
- Previous symbol:
- PDB3, OSIL
- Synonyms:
- p62, p60, p62B, A170
- Chromosome:
- 5q35.3
- Locus Type:
- gene with protein product
- Date approved:
- 2000-06-13
- Date modifiied:
- 2019-03-07
Related products to: SQSTM1 Antibody (Center S207) Blocking Peptide
Related articles to: SQSTM1 Antibody (Center S207) Blocking Peptide
- Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by progressive loss of upper and lower motor neurons, yet it is unknown whether diverse genetic risks converge on a shared pathogenic pathway. Here we identify a common mechanism linking TBK1 insufficiency to inflammatory cell death across distinct ALS genotypes. Whole-exome sequencing of 8 familial ALS patients revealed that 3 of them carried pathogenic variants, namely TBK1 (R573H), TDP-43 (G298V)/GARS (I44M)/SETX (L1304W), or SOD1 (G94R)/SQSTM1 (G262R). Motor neurons differentiated from patient iPSCs, despite their different mutations, consistently exhibited axonal and neurite shortening, reduced TBK1 activity, increased phosphorylated TDP-43 with cytoplasmic aggregation, and co-assembly of caspase-8/7 with the pyroptosis executor gasdermin E (GSDME). Neuroinflammatory stimulation further enhanced caspase-8/7 and GSDME cleavage, increased RIPK1 phosphorylation and suppressed TBK1 activation in patient-derived motor neurons, whereas TBK1 knockdown in SH-SY5Y cells recapitulated these phenotypes. Furthermore, a generated humanized TBK1 R573H knock-in mouse developed progressive motor deficits accompanied by spinal motor-neuron loss, neuroinflammation, and TDP-43 pathology. In these mice, LPS challenge produced increased cleavage of GSDME and caspase-7, along with a marked upregulation of p-TDP-43 expression in vulnerable neurons. Together, these data identify TBK1 loss of function as an upstream driver of a caspase-8/7-GSDME pyroptotic program in motor neurons and provide a mechanistic bridge between neuroinflammation, TDP-43 proteinopathy, and neurodegeneration. Targeting the TBK1-caspase-GSDME axis may offer a tractable strategy for disease modification in ALS. - Source: PubMed
Publication date: 2026/09/21
Qi ZihanLiu XueheGeng LuLiu XiaoniDong SiqiLi JiatongHua WenyiDeng YifanLai SuomeiWang YuhaoXia YuhongHan YuyunZhu DongqingZhang XiangLi JinsongLi JixiChen Xiangjun - Hyperbilirubinemia arises from disrupted bilirubin homeostasis, which can lead to bilirubin encephalopathy (BE). Unconjugated bilirubin (UCB)-induced neuroinflammation plays a pivotal role in the pathogenesis and progression of BE. - Source: PubMed
Publication date: 2026/09/21
Liu ShashaLi LingLi JingLi SiyuChen JiaqiSun QianZhang YanHua Ziyu - We describe the generation and characterization of two induced pluripotent stem cell (iPSC) lines: one derived from an ALS patient carrying the heterozygous SQSTM1 c.1175C > T (p.Pro392Leu) mutation, identified as cell line 047, and another one (cell line 053) derived from a patient carrying the heterozygous TBK1 c.1760 + 4_1760 + 7delAGTA likely pathogenic variant, predicted to result in a premature stop codon +, together with the ERBB4 c.2402A > T (p.His801Leu) variant of unknown significance (VUS). - Source: PubMed
Publication date: 2026/09/15
Santangelo SerenaCasarotto VeronicaCampelli LorenzoLeoni SerenaCabras SaraMatteoni EnricoMarco Giovanni DeCanosa AntonioBasso ManuelaBonetto ValentinaTrojisi FrancescaBossolasco PatriziaSilani VincenzoChiò AdrianoTicozzi NicolaCalvo AndreaRatti Antonia - This study establishes and characterizes a novel experimental mouse model to evaluate infection under type 1 diabetic conditions, providing a comprehensive description of the associated clinicopathological and immunometabolic alterations. - Source: PubMed
Publication date: 2026/09/03
Oliveira Mariana AraujoTavares Ywa Perpétuo Socorro TodaAlbuquerque Renata ChavesMigliorini SileneAlmeida Sandro RogérioMartins Joilson O - Atherosclerosis is a chronic vascular disease characterized by the accumulation of lipid-filled foam cells in the arterial wall. Macrophage-derived foam cells play a key role in promoting plaque formation. α-Tocopherol (α-TOC), a common dietary antioxidant, is thought to have protective effects against atherosclerosis, but its specific mechanisms remain unclear. In this study, an foam cell model was established using oxidized low-density lipoprotein-induced macrophages to investigate the effects of α-TOC on foam cell formation and related mechanisms. Network pharmacology predicted that α-TOC may act through autophagy-related pathways, particularly the mTOR-mediated autophagy pathway. We demonstrated that α-TOC enhanced autophagic function in macrophages, as evidenced by increased expression of Beclin1, LC3-II, ATG5, and ATG12, along with reduced p62/SQSTM1 levels. Rapamycin reproduced, and 3-methyladenine opposed, the direction of the autophagic and mTOR/ULK1 changes observed with α-TOC, consistent with involvement of this pathway. These findings suggest that α-TOC reduces lipid accumulation and inhibits foam cell formation in macrophages by enhancing autophagic activity in association with reduced mTOR phosphorylation and increased ULK1 Ser555 phosphorylation. This study provides mechanistic insights into the potential anti-atherosclerotic effects of α-TOC at the cellular level, offering a theoretical basis for its further investigation as a therapeutic candidate for atherosclerosis. - Source: PubMed
Publication date: 2026/09/16
Kuang LuWu LimeiLiu ZenghuiZhao JiaxingChen QijunYin HuayuLiu XuehuiLiu DabinWu Shaoguo