Ask about this productRelated genes to: LONRF2 antibody
- Gene:
- LONRF2 NIH gene
- Name:
- LON peptidase N-terminal domain and ring finger 2
- Previous symbol:
- -
- Synonyms:
- FLJ45273, RNF192
- Chromosome:
- 2q11.2
- Locus Type:
- gene with protein product
- Date approved:
- 2005-08-19
- Date modifiied:
- 2014-11-18
Related products to: LONRF2 antibody
Related articles to: LONRF2 antibody
- - Source: PubMed
Wood Heather - Protein misfolding is a major factor of neurodegenerative diseases. Post-mitotic neurons are highly susceptible to protein aggregates that are not diluted by mitosis. Therefore, post-mitotic cells may have a specific protein quality control system. Here, we show that LONRF2 is a bona fide protein quality control ubiquitin ligase induced in post-mitotic senescent cells. Under unperturbed conditions, LONRF2 is predominantly expressed in neurons. LONRF2 binds and ubiquitylates abnormally structured TDP-43 and hnRNP M1 and artificially misfolded proteins. Lonrf2 mice exhibit age-dependent TDP-43-mediated motor neuron (MN) degeneration and cerebellar ataxia. Mouse induced pluripotent stem cell-derived MNs lacking LONRF2 showed reduced survival, shortening of neurites and accumulation of pTDP-43 and G3BP1 after long-term culture. The shortening of neurites in MNs from patients with amyotrophic lateral sclerosis is rescued by ectopic expression of LONRF2. Our findings reveal that LONRF2 is a protein quality control ligase whose loss may contribute to MN degeneration and motor deficits. - Source: PubMed
Publication date: 2023/07/20
Li DanJohmura YoshikazuMorimoto SatoruDoi MiyukiNakanishi KeikoOzawa ManabuTsunekawa YujiInoue-Yamauchi AkaneNaruse HiroyaMatsukawa TakashiTakeshita YukioSuzuki NaokiAoki MasashiNishiyama AyumiZeng XinKonishi ChiekoSuzuki NarumiNishiyama AtsuyaHarris Alexander StephenMorita MarikoYamaguchi KiyoshiFurukawa YoichiNakai KentaTsuji ShojiYamazaki SatoshiYamanashi YujiShimada ShoichiOkada TakashiOkano HideyukiToda TatsushiNakanishi Makoto - - Source: PubMed
Youssef Mohieldin M MPark Jeehye - This study was designed to identify the differentially expressed miRNAs (DEMs) and genes (DEGs) in metastatic cervical cancer using bioinformatic tools. In this study, fifty-seven DEMs (48 downregulated and 9 upregulated) were identified, among which miR-4459 and miR-3195 expression was negatively associated with overall survival of cervical cancer patients. Then, 476 target DEGs were determined, and protein-protein interaction (PPI) network was constructed. Seventeen hub genes (, , , , , , , , , , , , , , , , and ) were finally selected to construct the miRNA-hub gene network. Overall, our study discovered the key miRNAs and mRNAs related to lymph node metastasis (LNM) in cervical cancer, which helps discover candidate therapeutic targets for cervical cancer. - Source: PubMed
Publication date: 2023/05/11
Ding YishanWu XiaorongYang Xiaofeng - The LONRF family of proteins consists of three isozymes, LONRF1-3, which harbors RING (really interesting new gene) domain and Lon substrate binding domain. We have recently identified LONRF2 as a protein quality control ubiquitin ligase that acts predominantly in neurons. LONRF2 selectively ubiquitylates misfolded or damaged proteins for degradation. LONRF2-/- mice exhibit late-onset neurological deficits. However, the physiological implications of other LONRF isozymes remain unclear. Here, we analysed Lonrf1 expression and transcriptomics at the single-cell level under normal and pathological conditions. We found that Lonrf1 was ubiquitously expressed in different tissues. Its expression in LSEC and Kupffer cells increased with age in the liver. Lonrf1high Kupffer cells showed activation of regulatory pathways of peptidase activity. In normal and NASH (nonalcoholic steatohepatitis) liver, Lonrf1high LSECs showed activation of NF-kB and p53 pathways and suppression of IFNa, IFNg and proteasome signalling independent of p16 expression. During wound healing, Lonrf1high/p16low fibroblasts showed activation of cell growth and suppression of TGFb and BMP (bone morphogenetic protein) signalling, whereas Lonrf1high/p16high fibroblasts showed activation of WNT (wingless and Int-1) signalling. These results suggest that although Lonrf1 does not seem to be associated with senescence induction and phenotypes, LONRF1 may play a key role in linking oxidative damage responses and tissue remodelling during wound healing in different modes in senescent and nonsenescent cells. - Source: PubMed
Li DanWang Teh-WeiAratani SaeOmori SatotakaTamatani MahoJohmura YoshikazuNakanishi Makoto