Ubikitinasyon Bozukluk Hastalıkları
Kitapta bölümler > Diğer · 2021
Yazarlar: Raziye Burcu GÜVEN, Afig BERDELİ
Genetik Otoinflamatuar Hastalıklar Tanı Yöntemleri ve Tedavilerinde Yenilikler, Diğer, Türkiye Klinikleri Yayınevi, Tanınmış Ulusal, Ulusal, 2021, ss 69-76, Türkiye, Ankara
Özet
<p><span style="color: rgb(34, 34, 34); font-family: Verdana, sans-serif; font-size: 11px; text-align: justify;">Autoinflammatory diseases are caused by mutations in genes that regulate the innate immunity. The NF-kB pathway has a critical role for inflammatory and immune responses, and abnormal NF-kB signaling is implicated autoimmune and chronic inflammatory disorders. NF-kB is regulated by different post-translational modifications, including phosphorylation and multiple ubiquitinations. Ubiquitination is a post-translational modification that regulates multiple cellular processes. Linear ubiquitin chain assembly complex (LUBAC) plays an important role in the activation of canonical NF-kB pathway. Furthermore, specific deubiquitinases, inhibit LUBAC-induced NF-kB activation. Functional impairments of these factors are associated with various disorders, including autoinflammation and immunodeficiencies. In this chapter, we discuss the mechanisms of linear ubiquitin chain generation and fragmentation by their specific enzymes, and review the current literature in terms of their biological functions and association with diseases.</span><br style="color: rgb(34, 34, 34); font-family: Verdana, sans-serif; font-size: 11px; text-align: justify;"><br style="color: rgb(34, 34, 34); font-family: Verdana, sans-serif; font-size: 11px; text-align: justify;"><b style="color: rgb(34, 34, 34); font-family: Verdana, sans-serif; font-size: 11px; text-align: justify;">Keywords</b><span style="color: rgb(34, 34, 34); font-family: Verdana, sans-serif; font-size: 11px; text-align: justify;">: Hereditary autoinflammatory diseases; polyubiquitin; NF-kappa B; deubiquitinating enzymes</span><br></p>