For the C1703A and C1706A mutations in Ubr1CA-KI, it is a TG to GC mutation in both Cys codons. For the C1128D mutation in Ubr1CD-KI, it is a TGC to AGC mutation for the Cys to Ser mutations. For Ubr1KO, we deleted the region from exon 6 to exon 10 in the Ubr1 gene locus. The nucleotide sequence of the deleted region is below. ctgggagcctgcttggcctgcagctgcttgaagcgctcgatggtccacagcacaaagtcataatgagcctccaactggccgggtaataggtaataaaataataaaacttttggaactttgaacttttagggattagacataccctcgggcagcgagcgagctcttccaacttctccagaattccgtactcctgggaacgctcgtagaagctcaagaagggatagtgctcgctaagctcctcctgaatggcataacccagcaggtgaaggacctgtagggaaataaacgaattgcaatttgaaaagaattgaaattttgttctaatgtaatacaaataccttttgaagatgcgattcggtaaagctgcgactgtaagcatttaaagccctttccataatcaagctgcagatgttgaggaacacagggcactgcaggatgttggccatgggtctagaatagattaggagattagagattgtatggattaagacatgatctctttagactcacgtgaaggctggtgttagttttggcaacattggtggcgggcagcagacaagctgcttcttggcctttcggcgctcccgttgcagttcctcggccttggacttgtcctctttggtgtagtgatagaagtacatgttgaactccttaagcaaatgttcctttagttcgtacacccccttgctgtcggctccaacgggtttcttgaacacggcaaccgtgttgataacttcctcgaaaacgttgtcgctatttccgctattgccatctggcaaggcgcgactcaactccgagtgtgagtatggcttgatacacaacagctggatgatctccttgcgcaggcgatcctcttcggtcaccatcgaaacgccaggcatccaacgttctccaatgatcacgatcagaagttccaggaactcgtcaaagattgacgcctctcgaatgatatcatcgttcatttcattacccgacaatagggaccagtagttttcctgtaaccaggggatcgtgttgaacctattcaacacatggatcaggaactcgttgctctccattagggaggcgcctatctgcaggcaggctatgtccctgtccagcatctccacgcggcaacgtacatttcggtagaagtagagctgatgcagcagggtgtaaccattgcggcgccacagtcctgcgcccacctgggcgatcattgcttgggtgcacaaaaccggttctataatctccctgggcgttagcttaatgctcaacgcttctgtttccgtctgcagcccgtcgtacgttaaatcgtgggcacccaagtgcagatagattccggcgaagaaacgcgacaaaggcaagtggatggataccggctgcactgatatgtcgtaaactagacagttggctacatggtccgccacttttttcggctgcatgaccttctcgccacccacaataaagctgttgctcaccagcgcccgcattgtcatcttgtagagcttacgcagcagcttcacatcgccggacgcccaatcaatcacctgcgagatggtcgtggccagcttgatgtgcaggttgaaggcgcactcccactctggctcgtagtccatatgttggcccgtctgtctggtcatcgactccatgccctgcatcacgtttagaacgcgcatcaaagcccggcagcctgtgtgttacaaatggggtaaatgagcttaaatggcttgcaaattcaatatgttgccggtgcctaccttctaggaaaccgtttcgtaaatcattgctcagcacatccggctttaggctgagcaggtatcgcagatcgtagaggatgtagttagcccgcttgaagaaggtgaggctggcgatgttcttgctaaagtgcagcgtcttgttacgaatgaacttctcgatggcaacgtggtagaacgtgtgcagcagcttgtcaaagatgccctcatgagcgatcagatgatgggctatgctgggcaccgtgaacagctggacactgagcgacacaatcgaaaaggcgtgatcatgatcatcgctgataaagtcctccacaatagtggcgtagcgtcgcgaaaactcctgggctaggatcattttgttgtcgtactccataagcataccagagatcaggaggcggtgccagcaggtgcgcgccgtcttccaaagcttcacgtcgtactcaaggatgtgccggatgcagaatgtctcctgcttgcgctgcaccaactcggagaacgtcttccggaagaggtagtgtcgcaccaggaactcctggaaccagccgagaagctgcagggcaaactgctgacaggccacggccccgatgtgcagcaccgatgtcctgagtgactgattgttgcgtgccgtcgacaccag