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RNA
Published in Paul Pumpens, Single-Stranded RNA Phages, 2020
Then, there was some similarity between the proposed secondary structure for the 5′ leader sequence and the cloverleaf structure of tRNA. This similarity was even more pronounced when the complement, the 3′ end of the minus strand, was compared to tRNA. It should be emphasized that the minus strand of the MS2 RNA also ended with …CCA (Fiers 1975), as did the MS2 RNA plus strand and the Oβ RNA plus and minus strands (Weber and Weissmann 1970). In this connection with the tRNA-like structure, it is necessary to mention here that the elongation factors Tu-Ts, which functioned in the protein synthesis machinery as carriers of charged tRNA, have been identified as components of the Qβ replicase complex (Blumenthal et al. 1972) and could be a part of the replicase complex of the group I phages (Fedoroff and Zinder 1971). This aspect of the RNA phage function will be unveiled systematically in Chapter 13. Moreover, tRNA nucleotidyltransferase added back CMP and AMP to genome fragments of the RNA phages MS2, R17, and Qβ, which have been obtained by incubation with snake venom phosphodiesterase, in conditions in which the enzyme remained highly specific of CCA-deprived tRNAs (Prochiantz et al. 1975). These observations might indicate that the phage RNAs contained certain features probably present in all tRNAs and recognized by the transferase.
Hematopoietic stem cell transplantation in systemic autoinflammatory diseases - the first one hundred transplanted patients
Published in Expert Review of Clinical Immunology, 2022
Sara Signa, Gianluca Dell’Orso, Marco Gattorno, Maura Faraci
Patients with SIFD (Sideroblastic anemia with B-cell Immunodeficiency, periodic fever, and Developmental delay) present loss of function (LOF) homozygous or compound heterozygous mutations in the TRNT1 gene, coding for tRNA nucleotidyltransferase cytidine-cytidine- adenosine-adding 1, implicated in tRNAs maturation. The clinical picture is characterized by an early onset systemic inflammation with musculoskeletal and mucocutaneous alterations, associated with developmental delay [26,27]. Activation of UPR causes an abnormal B cell maturation leading to immunoglobulin (Ig) deficiency [28]. Anti-tumor necrosis factor (TNF) treatments are more effective than IL-1 inhibitors in these patients [7]. HSCT can correct the immunological and hematological defect, with resolution of inflammation and independence from transfusions and IVIG (intravenous immunoglobulin) replacement [26,27,29]. However, the neurologic manifestations possibly associated with this condition do not benefit from HSCT [29]: in fact, development of pigmentary retinitis in one patient was reported some years after HSCT [26]. At present, only four transplanted patients are described and one of them died soon after transplant due to a pulmonary hemorrhage [27,29] (Table 2).