Web001811 B6EiC3Sn a / A - Otc spf-ash /J The Otc spf-ash mouse model of chronic hyperammonemia is useful to study acute metabolic decompensation with … WebFeb 3, 2024 · The spf ash mouse model carries a variant (c.386G>A, p.Arg129His) that is also found in patients. Male spf ash mice have a mild biochemical phenotype with low OTC activity (5%‐10% of wild‐type), resulting in elevated urinary orotic acid but no hyperammonemia.

Table 1. OTC deficient patients with the c.386G>A mutation.

WebOtc spf-ash: Name: ornithine transcarbamylase; abnormal skin and hair: MGI ID: MGI:1856179: Synonyms: spf ash, spf-ash: Gene: ... Mouse strains and cell lines available … WebJul 7, 1996 · Kiwaki et al. report the adenoviral-mediated transfer of the ornithine transcarbamylase (OTC) gene. Using the CAG promoter, enzyme activity equal to that of … paint cartridge roller wholesale distributor

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WebThe spf/ash mouse model of ornithine transcarbamylase (OTC) deficiency, a severe urea cycle disorder, is caused by a mutation (c.386G>A; p.R129H) in the last nucleotide of exon … WebBibTeX @MISC{Wang_sustainedcorrection, author = {Lili Wang and Huan Wang and Hiroki Morizono and Peter Bell and David Jones and Jianping Lin and Deirdre Mcmenamin and … WebApr 1, 2024 · Compared to our previous gene therapy study, vector GC in OTC-KO Het females were about 10-fold lower than they were in male OTC spf ash mice treated with the same doses of this vector at similar time points [17]. This is consistent with previous reports on gender differences in AAV gene transfer in murine liver [6], [30], [31]. substitute for black treacle in baking