DNA polymerase-α regulates the activation of type I interferons through cytosolic RNA:DNA synthesis

The causative mechanism for the immunodeficiency and autoinflammatory disease XLPDR is unknown. Burstein and colleagues show that XLPDR is caused by disruption of POLA1 , which encodes a DNA polymerase subunit; this, in turn, leads to dysregulated production of type I interferons. Aberrant nucleic a...

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Veröffentlicht in:	Nature immunology 2016-05, Vol.17 (5), p.495-504
Hauptverfasser:	Starokadomskyy, Petro, Gemelli, Terry, Rios, Jonathan J, Xing, Chao, Wang, Richard C, Li, Haiying, Pokatayev, Vladislav, Dozmorov, Igor, Khan, Shaheen, Miyata, Naoteru, Fraile, Guadalupe, Raj, Prithvi, Xu, Zhe, Xu, Zigang, Ma, Lin, Lin, Zhimiao, Wang, Huijun, Yang, Yong, Ben-Amitai, Dan, Orenstein, Naama, Mussaffi, Huda, Baselga, Eulalia, Tadini, Gianluca, Grunebaum, Eyal, Sarajlija, Adrijan, Krzewski, Konrad, Wakeland, Edward K, Yan, Nan, de la Morena, Maria Teresa, Zinn, Andrew R, Burstein, Ezra
Format:	Artikel
Sprache:	eng
Schlagworte:	13/106 13/21 13/31 14/1 14/63 38/23 38/47 38/90 45/22 631/1647/664/2089 631/250/249/1313 631/250/262/2106 631/45/147 692/699/249/2512 Base Sequence Biomedicine Cells, Cultured Cytosol - metabolism Development and progression DNA - biosynthesis DNA - genetics DNA Polymerase I - genetics DNA Polymerase I - metabolism DNA polymerases Family Health Female Fibroblasts - cytology Fibroblasts - metabolism Gene Expression Profiling Genetic aspects Genetic Diseases, X-Linked - genetics Genetic Diseases, X-Linked - metabolism HEK293 Cells HeLa Cells Humans Immune response Immunoblotting Immunology Infectious Diseases Interferon Type I - metabolism Male Microscopy, Confocal Mutation Oligonucleotide Array Sequence Analysis Pedigree Pigmentation disorders Pigmentation Disorders - genetics Pigmentation Disorders - metabolism Properties Reverse Transcriptase Polymerase Chain Reaction RNA - biosynthesis RNA - genetics
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