Unc93b1S282A mice carry a CRISPR/Cas9 generated serine to alanine change at amino acid 282 (S282) of the unc-93 homolog B1, TLR signaling regulator (Unc93b1) gene (cloned from the second start site of the Unc93b1 gene [MEVEPPLY]), analogous to amino acid 282 (S282) in humans. Since this gene has two start sites, this amino acid would be S303A when cloned from the first alternative start site (MKEVPT). Unc93b1 encodes a trafficking chaperone required for the transport of some toll-like receptors (TLRs) from the endoplasmic reticulum to endosomes where they can engage pathogen nucleotides and activate signaling cascades. Release of TLR9 from Unc93b1, which only occurs in endosomes, is required for activation of the receptor and subsequent ligand binding and signal transduction. Mutations in Unc93b1, such as the one present in this strain, cause misregulation of TLR trafficking and can lead to the onset of some autoimmune and autoinflammatory diseases. Homozygous mice are viable and fertile.

The use of this mouse model is subject to the terms and conditions of the Limited Use Label License from Caribou Biosciences, Inc.

The use of this mouse strain or other product (the "Licensed Product") is subject to the terms and conditions of the Limited License from The Broad Institute.

Unc93b1S282A has a serine to alanine change in the unc-93 homolog B1, TLR signaling regulator (Unc93b1) gene, analogous to amino acid 282 (S282) in humans. This strain useful when studying the role of Unc93b1 in the regulation of toll-like receptor activation.

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