Inborn errors of TLR3-dependent IFN-a/b-and-l-mediated immunity in the central nervous system (CNS) can underlie herpes simplex virus 1 (HSV-1) encephalitis (HSE). The respective contributions of IFN-a/b and-l are unknown. We report a child homozygous for a genomic deletion of the entire coding sequence and part of the 3'UTR of the last exon of IFNAR1, who died from HSE at the age of two years. An older cousin died following vaccination against measles, mumps and rubella at 12 months of age, and another 17-year-old cousin homozygous for the same variant has had other, less severe viral illnesses. The encoded IFNAR1 protein is expressed on the cell surface but is truncated and cannot interact with the tyrosine kinase TYK2. The patient's fibroblasts and EBV-B cells did not respond to IFN-a2b or IFN-b, in terms of STAT1, STAT2 and STAT3 phosphorylation, or the genome-wide induction of IFN-stimulated genes. The patient's fibroblasts were susceptible to viruses, including HSV-1, even in the presence of exogenous IFN-a2b or IFN-b. HSE is therefore a consequence of inherited complete IFNAR1 deficiency. This viral disease occurred in natural conditions, unlike those previously reported in other patients with IFNAR1 or IFNAR2 deficiency. This experiment of Nature indicates that IFN-a/b are essential for anti-HSV-1 immunity in the CNS.
Haedeul' is a high-quality rice variety with early maturing that adapts to the central region of Korea. This cultivar was crossed between 'Gopum', which has excellent taste, and 'Hoban', which has a low viviparous germination rate and is moderately tolerant of cold, by the rice breeding team of the Department of Central Area Crop Science, NlCS, RDA, in 2017. In early-transplanting cultivation, the heading date of 'Haedeul' was July 24 in central area of Korea, which was seven days later than that of 'Jopyeong'. 'Haedeul' had a culm length of 75 cm and 99 spikelets per panicle. The viviparous germination rate of 'Haedeul' was 7.7%. It showed resistance to blast and bacterial blight (K1, K2, K3 race), but was susceptible to the K3a race of bacterial blight, stripe virus, dwarf and black streak dwarf viruses, and plant-hoppers. The milled rice of this variety exhibited a translucent and medium short grain shape. The cooked rice grains of 'Haedeul' had an excellent palatability index (0.61) and lower amylose content (18.0%) than that of 'Jopyeong'. The grain milling characteristics were better than those of 'Jopyeong', especially the head rice milling recovery ratio and head rice ratio (94.9%). 'Haedeul' milled rice productivity averaged 5.32 MT/ha at five sites under ordinary cultivation (Registration No. 7681).
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