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RMRP encodes a non-coding RNA forming the core of the RNase MRP ribonucleoprotein complex. Mutations cause Cartilage Hair Hypoplasia (CHH), characterized by skeletal abnormalities and impaired T cell activation. Yeast RNase MRP cleaves a specific site in the pre-ribosomal RNA (pre-rRNA) during ribosome synthesis. CRISPR-mediated disruption of RMRP in human cells lines caused growth arrest, with pre-rRNA accumulation. Here, we analyzed disease-relevant primary cells, showing that mutations in RMRP impair mouse T cell activation and delay pre-rRNA processing. Patient-derived human fibroblasts with CHH-linked mutations showed similar pre-rRNA processing delay. Human cells engineered with the most common CHH mutation (70 AG in RMRP ) show specifically impaired pre-rRNA processing, resulting in reduced mature rRNA and a reduced ratio of cytosolic to mitochondrial ribosomes. Moreover, the 70 AG mutation caused a reduction in intact RNase MRP complexes. Together, these results indicate that CHH is a ribosomopathy. Mutations in the non-coding RNA RMRP cause primary immunodeficiency. Robertson et al show that a disease-associated mutation in RMRP impairs pre-ribosomal RNA processing and reduces ribosome abundance, establishing this disorder as a ribosomopathy.

Infection with SARS-CoV-2 is expected to result in substantial reorganization of host cell RNA metabolism. We identified 14 proteins that were predicted to interact with host RNAs or RNA binding proteins, based on published data for SARS-CoV and SARS-CoV-2. Here, we describe a series of affinity-tagged and codon-optimized expression constructs for each of these 14 proteins. Each viral gene was separately tagged at the N-terminus with Flag-His 8 , the C-terminus with His 8 -Flag, or left untagged. The resulting constructs were stably integrated into the HEK293 Flp-In T-REx genome. Each viral gene was expressed under the control of an inducible Tet-On promoter, allowing expression levels to be tuned to match physiological conditions during infection. Expression time courses were successfully generated for most of the fusion proteins and quantified by western blot. A few fusion proteins were poorly expressed, whereas others, including Nsp1, Nsp12, and N protein, were toxic unless care was taken to minimize background expression. All plasmids can be obtained from Addgene and cell lines are available. We anticipate that availability of these resources will facilitate a more detailed understanding of coronavirus molecular biology.

Purpose This paper aims to explore the delayed, then accelerated, internationalisation of an emerging multinational enterprise (EMNE), with a particular focus on the media technology sector, and how it exploited complementarities between emerging markets. Design/methodology/approach The research is qualitative in nature and focuses on the expansion of a South African media technology EMNE case study that has a footprint in over 130 countries and has one of the largest market capitalisations of any media company outside the USA and China. Findings EMNEs have unique capabilities in navigating uncertain institutional environments in emerging markets and are able to capitalise upon the institutional complementarities between their home and host countries. This may facilitate the recognition of market opportunities and the harnessing of new technologies to meet these opportunities in complementary markets for accelerated internationalisation. Practical implications EMNEs must capitalise upon the institutional complementarities between home and host country locations and use this to take advantage of identified market opportunities. This creates the possibility for a process of accelerated internationalisation. New technologies are creating particular market opportunities in emerging markets which can be exploited by EMNEs. Originality/value The authors provide a framework which illustrates how an EMNE can exploit complementarities between emerging markets to identify market opportunities, capitalise upon institutional similarities and harness new technologies in the process.

PurposeThis paper aims to explore the delayed, then accelerated, internationalisation of an emerging multinational enterprise (EMNE), with a particular focus on the media technology sector, and how it exploited complementarities between emerging markets.Design/methodology/approachThe research is qualitative in nature and focuses on the expansion of a South African media technology EMNE case study that has a footprint in over 130 countries and has one of the largest market capitalisations of any media company outside the USA and China.FindingsEMNEs have unique capabilities in navigating uncertain institutional environments in emerging markets and are able to capitalise upon the institutional complementarities between their home and host countries. This may facilitate the recognition of market opportunities and the harnessing of new technologies to meet these opportunities in complementary markets for accelerated internationalisation.Practical implicationsEMNEs must capitalise upon the institutional complementarities between home and host country locations and use this to take advantage of identified market opportunities. This creates the possibility for a process of accelerated internationalisation. New technologies are creating particular market opportunities in emerging markets which can be exploited by EMNEs.Originality/valueThe authors provide a framework which illustrates how an EMNE can exploit complementarities between emerging markets to identify market opportunities, capitalise upon institutional similarities and harness new technologies in the process.

Assad's army is seen fleeing out of Syria crossing into Iraq asrebel forces said they're at the gates of Damascus. NYPD is in Atlantaafter suspect in UnitedHealthcare CEO shooting is seen riding the bus,while divers searched Central Park Lake for evidence. Trump isreturning to world stage as Notre Dame Cathedral reopened its doorsfive years after devastating fire. President-elect Trump once againexpressing confidence in his pick, Pete Hegseth to lead the Departmentof Defense. GUESTS: Chris Swecker, Alexandra de Hoop Scheffer, Jasmine Wright

President Biden on his state visit portion of his trip toFrance with French President Macron at the welcome parade ceremony atthe Arc de Triomphe. IDF says it has rescued four hostages who weretaken from the Nova Music Festival on October 7th, among the freedhostages is Noa Argamani. Today was Benny Gantz's self-imposeddeadline to resign if Prime Minister Benjamin Netanyahu did notpresent a new plan for the war, but he postponed his announcement,apparently, due to the rescue of these four Israeli hostages. FrenchPresident Emmanuel Macron hosted President Joe Biden and First LadyJill Biden for an official state visit in Paris, with the agenda onglobal security issues, such as the conflicts in Ukraine and Gaza, andalso to commemorate the 80th anniversary of D-Day.

President Biden attends the wreath-laying ceremony at thePentagon 9/11 memorial, joined by First Lady Jill Biden, VicePresident Kamala Harris, first gentleman Doug Emhoff, and DefenseSecretary Lloyd Austin. As Americans mark 20 years since 9/11, it willbe the first time without any U.S. troops on the ground inAfghanistan. Ann Compton, former ABC News White House correspondent,discusses 9/11. GUESTS: Ali Soufan, Ann Compton, Terry Strada