Ngly1(N-glycanase 1) encodes an enzyme that catalyzes the deglycosylation of misfolded N-linked glycoproteins. Mutations in this gene are associated with Congenital Disorders of Glycosylation Type 1v, an autosomal recessive multi-system disorder characterized by global developmental delay, hypotonia, abnormal involuntary movements and poor tear production. Ngly1cKOex4 mice were created using CRISPR/cas9 genome editing to insert loxP sites flanking exon 4. Mice heterozygous for the mutation are viable and fertile. When bred to mice that express tissue-specific Cre recombinase, resulting offspring are expected to have exon 4 deleted in cre-expressing tissues. To date (September 2021), full characterization is in progress and deletion of the floxed allele has not been confirmed. As the mice are characterized, we will modify the strain description.

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Ngly1cKOex4 is a CRISPR/cas9 generated mutant that contains loxP sites flanking exon 4 of the N-glycanase 1 gene. &nbsp;These mice may be useful in generating conditional mutations to study Congenital Disorders of Glycosylation Type 1v.

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