LEADER 04357nam 2200469z- 450 001 9910136792803321 005 20210212 035 $a(CKB)3710000000631056 035 $a(oapen)https://directory.doabooks.org/handle/20.500.12854/58041 035 $a(oapen)doab58041 035 $a(EXLCZ)993710000000631056 100 $a20202102d2016 |y 0 101 0 $aeng 135 $aurmn|---annan 181 $ctxt$2rdacontent 182 $cc$2rdamedia 183 $acr$2rdacarrier 200 00$aRegulation of Tissue Responses: The TWEAK/Fn14 Pathway and other TNF/ TNFR Superfamily Members that Activate Noncanonical NFkB Signaling 210 $cFrontiers Media SA$d2016 215 $a1 online resource (201 p.) 225 1 $aFrontiers Research Topics 311 08$a2-88919-700-X 330 $aThe immune system mediates tissue responses under both physiological and pathological conditions, impacting the inflammatory, fibrogenic and regenerative components. In addition to various leukocyte subsets, it is now recognized that epithelial, endothelial and other non-hematopoietic tissue cell types actively contribute to the interplay shaping tissue responses. Further understanding the molecular pathways and mechanisms mediating these tissue responses is of great interest. In the past decade, TNF-like weak inducer of apoptosis (TWEAK) and its receptor, FGF-inducible molecule-14 (Fn14), members of the TNF/TNFR superfamily, have emerged as a prominent molecular axis regulating tissue responses. Generally leukocyte-derived, TWEAK signals through Fn14 which is highly induced in injured and diseased tissues on the surface of parenchymal, stromal and progenitor cells, thereby orchestrating a host of tissue-shaping responses, including inflammation, angiogenesis, cell proliferation or death, and the regulation of progenitor cells. Compelling preclinical results indicate that whereas transient TWEAK/Fn14 activation promotes productive tissue responses after acute injury, excessive or persistent TWEAK/Fn14 activation drives pathological tissue responses, leading to progressive damage and degeneration in target organs of injury, autoimmune and inflammatory diseases and cancer. Given that the highly inducible pattern of Fn14 expression is well conserved between mouse and man, the role of TWEAK/Fn14 in human disease is an area of intense investigation. Recent findings have also begun to shed light on how the TWEAK/Fn14 pathway fits into the immune network, interplaying with other well-established pathways, including TNFa, IL-17, IL-13 and TGFb, in regulating tissue responses. The noncanonical nuclear factor kB (NF?B) pathway plays a role in immunity and disease pathologies and appears to be activated by only a subset of TNF/ TNFR superfamily members. Of the various signaling pathways downstream of TWEAK/Fn14, particular attention has been placed on the noncanonical NF?B pathway given that given that TWEAK induces acute activation of canonical NF?B but prolonged activation of noncanonical pathway. Thus dovetailing of the TWEAK/Fn14 axis with noncanonical NF?B pathway activation may be a key mechanism underlying tissue responses. Also of great interest is a deeper understanding of where, when and how tissue responses are regulated by other TNF/ TNFR superfamily members that can signal through noncanonical NF?B. This Research Topic issue will cover: 1. TWEAK/Fn14 pathway biology, role in tissue responses, injury, and disease pathogenesis 2. Role of noncanonical NFkB signaling cascade in tissue responses 3. Translational studies of relevance of TWEAK/Fn14 and noncanonical NFkB in human disease 4. Other TNF superfamily members' signaling through noncanonical NFkB in the regulation of tissue responses 5. Reviews and Perspectives on the above 517 $aRegulation of Tissue Responses 606 $aMedicine and Nursing$2bicssc 610 $aBAFFR 610 $aCD40 610 $aFn14 610 $aLTbR 610 $aNFkB 610 $aNoncanonical 610 $aRank 610 $aTNFR2 610 $aTWEAK 615 7$aMedicine and Nursing 700 $aLinda C. Burkly$4auth$01322466 702 $aJohn Silke$4auth 702 $aTimothy S. Zheng$4auth 906 $aBOOK 912 $a9910136792803321 996 $aRegulation of Tissue Responses: The TWEAK$93035039 997 $aUNINA LEADER 03104nam 2200601z- 450 001 9910557124503321 005 20210501 035 $a(CKB)5400000000040809 035 $a(oapen)https://directory.doabooks.org/handle/20.500.12854/68296 035 $a(oapen)doab68296 035 $a(EXLCZ)995400000000040809 100 $a20202105d2021 |y 0 101 0 $aeng 135 $aurmn|---annan 181 $ctxt$2rdacontent 182 $cc$2rdamedia 183 $acr$2rdacarrier 200 00$aJuvenile Onset Huntington's Disease 210 $aBasel, Switzerland$cMDPI - Multidisciplinary Digital Publishing Institute$d2021 215 $a1 online resource (102 p.) 311 08$a3-03943-811-5 311 08$a3-03943-812-3 330 $aThe Special Issue "Juvenile Onset Huntington's Disease" highlights the growing interest in understanding the unique aspects of this ultra-rare disorder. After decades of research, gene therapy trials are underway for Adult Onset Huntington's Disease (AOHD). However, patients with Juvenile Onset Huntington's Disease (JOHD) are often excluded from these efforts, leaving many questions regarding its phenomenology. The current issue includes seven articles spanning work on the difficult emotional experiences of parents of children with JOHD; a review of the clinical manifestations of JOHD; behavioral issues in JOHD; CAG repeat and age of motor onset; autonomic nervous system dysfunction; and abnormality in MRI metabolic markers. Finally, a review of the therapeutic advances is included, highlighting future possibilities of clinical trials in JOHD subjects. The HD community-patients, family members at-risk for HD, caregivers, health-care professionals and scientists-is keen on expanding our understanding of JOHD. In the flurry of research on AOHD, those with JOHD were seemingly 'left behind.' The study of patients who are afflicted early in life with HD has become imperative, with this Special Issue representing just the beginning of the required effort to address this urgent need. 606 $aMedicine and Nursing$2bicssc 610 $aautonomic 610 $abehavioral regulation 610 $aCAG 610 $aCAG repeat 610 $acaregivers 610 $acase series 610 $aearly-onset Huntington's disease 610 $aexecutive function 610 $aHuntington's disease 610 $ajuvenile Huntington's disease 610 $ajuvenile-onset Huntington's disease 610 $ajuvenile-onset Huntington's Disease 610 $amotor onset 610 $amutant huntingtin (mHTT) 610 $an/a 610 $aneurodegeneration 610 $aneuroimaging 610 $apediatric Huntington's disease 610 $apersonal experiences 610 $aT1-Rho 610 $atherapeutics 610 $atrinucleotide repeat disorder 615 7$aMedicine and Nursing 700 $aNopoulos$b Peggy C$4edt$01303358 702 $aNopoulos$b Peggy C$4oth 906 $aBOOK 912 $a9910557124503321 996 $aJuvenile Onset Huntington's Disease$93026942 997 $aUNINA