Central nervous system anomalies in 41 Chinese children incontinentia pigmenti. | Central nervous system anomalies in 41 Chinese children incontinentia pigmenti. Yin L, Li Z, Zhan W, Kang Y, Tian Q, Li D, Zhang H., Free PMC Article | 09/9/2024 |
NEMO/NF-kappaB signaling functions as a double-edged sword in PanIN formation versus progression to pancreatic cancer. | NEMO/NF-κB signaling functions as a double-edged sword in PanIN formation versus progression to pancreatic cancer. Tsesmelis M, Büttner UFG, Gerstenlauer M, Manfras U, Tsesmelis K, Du Z, Sperb N, Weissinger SE, Möller P, Barth TFE, Maier HJ, Chan LK, Wirth T., Free PMC Article | 08/15/2024 |
Familial recurrence of incontinentia pigmenti due to de novo pathogenic variants in the IKBKG gene. | Familial recurrence of incontinentia pigmenti due to de novo pathogenic variants in the IKBKG gene. Steffann J, De Oliveira Santos J, Zelbin AL, Hadj-Rabia S, Charbit-Henrion F, Petit F. | 07/26/2024 |
Dual regulation of NEMO by Nrf2 and miR-125a inhibits ferroptosis and protects liver from endoplasmic reticulum stress-induced injury. | Dual regulation of NEMO by Nrf2 and miR-125a inhibits ferroptosis and protects liver from endoplasmic reticulum stress-induced injury. Tak J, Joo MS, Kim YS, Park HW, Lee CH, Park GC, Hwang S, Kim SG., Free PMC Article | 03/22/2024 |
A conserved core region of the scaffold NEMO is essential for signal-induced conformational change and liquid-liquid phase separation. | A conserved core region of the scaffold NEMO is essential for signal-induced conformational change and liquid-liquid phase separation. DiRusso CJ, DeMaria AM, Wong J, Wang W, Jordanides JJ, Whitty A, Allen KN, Gilmore TD., Free PMC Article | 01/9/2024 |
Damaged mitochondria recruit the effector NEMO to activate NF-kappaB signaling. | Damaged mitochondria recruit the effector NEMO to activate NF-κB signaling. Harding O, Holzer E, Riley JF, Martens S, Holzbaur ELF., Free PMC Article | 09/11/2023 |
SARS-CoV-2 ORF3a positively regulates NF-kappaB activity by enhancing IKKbeta-NEMO interaction. | SARS-CoV-2 ORF3a positively regulates NF-κB activity by enhancing IKKβ-NEMO interaction. Nie Y, Mou L, Long Q, Deng D, Hu R, Cheng J, Wu J., Free PMC Article | 04/4/2023 |
TREM-1 induces pyroptosis in cardiomyocytes by activating NLRP3 inflammasome through the SMC4/NEMO pathway. | TREM-1 induces pyroptosis in cardiomyocytes by activating NLRP3 inflammasome through the SMC4/NEMO pathway. Yang Z, Pan X, Wu X, Lin Q, Chen Y, Cai S, Zhang Y, Mai Z, Ahmad N, Ma D, Deng L. | 03/22/2023 |
Extracellular vesicles-transferred SBSN drives glioma aggressiveness by activating NF-kappaB via ANXA1-dependent ubiquitination of NEMO. | Extracellular vesicles-transferred SBSN drives glioma aggressiveness by activating NF-κB via ANXA1-dependent ubiquitination of NEMO. Chen H, Chen X, Zhang Z, Bao W, Gao Z, Li D, Xie X, Zhou P, Yang C, Zhou Z, Pan J, Kuang X, Tang R, Feng Z, Zhou L, Zhu D, Yang J, Wang L, Huang H, Tang D, Liu J, Jiang L. | 12/10/2022 |
Human Genetic Diseases Linked to the Absence of NEMO: An Obligatory Somatic Mosaic Disorder in Male. | Human Genetic Diseases Linked to the Absence of NEMO: An Obligatory Somatic Mosaic Disorder in Male. Pescatore A, Spinosa E, Casale C, Lioi MB, Ursini MV, Fusco F., Free PMC Article | 05/14/2022 |
Genetically programmed alternative splicing of NEMO mediates an autoinflammatory disease phenotype. | Genetically programmed alternative splicing of NEMO mediates an autoinflammatory disease phenotype. Lee Y, Wessel AW, Xu J, Reinke JG, Lee E, Kim SM, Hsu AP, Zilberman-Rudenko J, Cao S, Enos C, Brooks SR, Deng Z, Lin B, de Jesus AA, Hupalo DN, Piotto DG, Terreri MT, Dimitriades VR, Dalgard CL, Holland SM, Goldbach-Mansky R, Siegel RM, Hanson EP., Free PMC Article | 04/30/2022 |
Fluoride induced down-regulation of IKBKG Gene expression inhibits hepatocytes senescence. | Fluoride induced down-regulation of IKBKG Gene expression inhibits hepatocytes senescence. Wu CX, Mao CY, Deng J, Zhang T, Zhao Y, Guan ZZ, Hu XX, Qi XL. | 03/5/2022 |
TRIM41 is required to innate antiviral response by polyubiquitinating BCL10 and recruiting NEMO. | TRIM41 is required to innate antiviral response by polyubiquitinating BCL10 and recruiting NEMO. Yu Z, Li X, Yang M, Huang J, Fang Q, Jia J, Li Z, Gu Y, Chen T, Cao X., Free PMC Article | 03/5/2022 |
Case Report: Analysis of Preserved Umbilical Cord Clarified X-Linked Anhidrotic Ectodermal Dysplasia With Immunodeficiency in Deceased, Undiagnosed Uncles. | Case Report: Analysis of Preserved Umbilical Cord Clarified X-Linked Anhidrotic Ectodermal Dysplasia With Immunodeficiency in Deceased, Undiagnosed Uncles. Inaba S, Aizawa Y, Miwa Y, Imai C, Ohnishi H, Kanegane H, Saitoh A., Free PMC Article | 02/19/2022 |
T Cell Impairment Is Predictive for a Severe Clinical Course in NEMO Deficiency. | T Cell Impairment Is Predictive for a Severe Clinical Course in NEMO Deficiency. Heller S, Kölsch U, Magg T, Krüger R, Scheuern A, Schneider H, Eichinger A, Wahn V, Unterwalder N, Lorenz M, Schwarz K, Meisel C, Schulz A, Hauck F, von Bernuth H. | 08/7/2021 |
Analysis of IKBKG/NEMO gene in five Japanese cases of incontinentia pigmenti with retinopathy: fine genomic assay of a rare male case with mosaicism. | Analysis of IKBKG/NEMO gene in five Japanese cases of incontinentia pigmenti with retinopathy: fine genomic assay of a rare male case with mosaicism. Haque MN, Ohtsubo M, Nishina S, Nakao S, Yoshida K, Hosono K, Kurata K, Ohishi K, Fukami M, Sato M, Hotta Y, Azuma N, Minoshima S. | 07/3/2021 |
SNW1 interacts with IKKgamma to positively regulate antiviral innate immune responses against influenza A virus infection. | SNW1 interacts with IKKγ to positively regulate antiviral innate immune responses against influenza A virus infection. Zhang Q, Liang T, Gu S, Ye Y, Liu S. | 07/3/2021 |
Molecular analysis of low-level mosaicism of the IKBKG mutation using the X Chromosome Inactivation pattern in Incontinentia Pigmenti. | Molecular analysis of low-level mosaicism of the IKBKG mutation using the X Chromosome Inactivation pattern in Incontinentia Pigmenti. Kawai M, Kato T, Tsutsumi M, Shinkai Y, Inagaki H, Kurahashi H., Free PMC Article | 06/26/2021 |
Antagonizing binding of cell cycle and apoptosis regulatory protein 1 (CARP-1) to the NEMO/IKKgamma protein enhances the anticancer effect of chemotherapy. | Antagonizing binding of cell cycle and apoptosis regulatory protein 1 (CARP-1) to the NEMO/IKKγ protein enhances the anticancer effect of chemotherapy. Venkatesh J, Sekhar SC, Cheriyan VT, Muthu M, Meister P, Levi E, Dzinic S, Gauld JW, Polin LA, Rishi AK., Free PMC Article | 12/12/2020 |
DNA-PK: gatekeeper for IKKgamma/NEMO nucleocytoplasmic shuttling in genotoxic stress-induced NF-kappaB activation. | DNA-PK: gatekeeper for IKKγ/NEMO nucleocytoplasmic shuttling in genotoxic stress-induced NF-kappaB activation. Medunjanin S, Putzier M, Nöthen T, Weinert S, Kähne T, Luani B, Zuschratter W, Braun-Dullaeus RC., Free PMC Article | 10/24/2020 |
The spectrum of pathologic manifestation in female incontinentia pigmenti adult patients caused by pathogenic variant in IKBKG has been reported. | Incontinentia pigmenti in adults. Scheuerle AE. | 08/12/2020 |
Incontinentia pigmenti (IP) is an X-linked dominant disease,generally lethal in males, caused by variants of theIKBKG/NEMOgene , which encodes for IKK-gamma/NEMO, essential for NF-kappaB activation | Clinical utility gene card: for incontinentia pigmenti. Fusco F, Pescatore A, Steffann J, Bonnefont JP, De Oliveira J, Lioi MB, Ursini MV., Free PMC Article | 07/18/2020 |
This study shows that a highly conserved central region of NEMO termed the intervening domain (IVD, amino acids 112-195) plays a key role in NEMO function. This articles proposes a model in which the IVD is required for a conformational change in NEMO that is necessary for its ability to direct phosphorylation of IkappaBalpha by IKK-beta. | A Central Region of NF-κB Essential Modulator Is Required for IKKβ-Induced Conformational Change and for Signal Propagation. Shaffer R, DeMaria AM, Kagermazova L, Liu Y, Babaei M, Caban-Penix S, Cervantes A, Jehle S, Makowski L, Gilmore TD, Whitty A, Allen KN., Free PMC Article | 06/6/2020 |
PAD4 exacerbates ischemic acute kidney injury and inflammation by promoting renal tubular NF-kappaB activity and inflammation via NEMO citrullination. | Peptidyl arginine deiminase-4 exacerbates ischemic AKI by finding NEMO. Rabadi MM, Han SJ, Kim M, D'Agati V, Lee HT., Free PMC Article | 02/1/2020 |
A recurrent deep intronic splicing mutation in IKBKG underlies a purely quantitative NEMO defect in males that is most severe in leukocytes and can be rescued by the inhibition of SRSF6 or CLK. | Rescue of recurrent deep intronic mutation underlying cell type-dependent quantitative NEMO deficiency. Boisson B, Honda Y, Ajiro M, Bustamante J, Bendavid M, Gennery AR, Kawasaki Y, Ichishima J, Osawa M, Nihira H, Shiba T, Tanaka T, Chrabieh M, Bigio B, Hur H, Itan Y, Liang Y, Okada S, Izawa K, Nishikomori R, Ohara O, Heike T, Abel L, Puel A, Saito MK, Casanova JL, Hagiwara M, Yasumi T., Free PMC Article | 12/21/2019 |