| Allele Name | transgene insertion 450B, Clotilde Lagier-Tourenne |
| Allele Type | Transgenic (Inserted expressed sequence, Humanized sequence) |
| Allele Synonym(s) | C9450B |
| Gene Symbol and Name | Tg(C9orf72*)450BLagi, transgene insertion 450B, Clotilde Lagier-Tourenne |
| Gene Synonym(s) | |
| Promoter | C9orf72, chromosome 9 open reading frame 72, human |
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| Expressed Gene | C9orf72, chromosome 9 open reading frame 72, human |
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| Strain of Origin | C57BL/6 x C3H |
| Chromosome | UN |
| General Note | Phenotypic Similarity to Human Syndrome in Orthologous Human Gene: (FRONTOTEMPORAL DEMENTIA AND/OR AMYOTROPHIC LATERAL SCLEROSIS 1; FTDALS1) |
| Molecular Note | The transgenic construct consists of a ~156 kbp human bacterial artificial chromosome (BAC) CH523-111K12 containing the human C9ALS patient-derived C9orf72 gene encoding a truncated short C9ORF72 isoform (222 aa; exons 1-5 with TAA site and polyA signal) with ~450 hexanucleotide repeat expansions (GGGGCC) in intron 1a. The BAC has ~140 kbp of transcriptionally-upstream (telomeric) flanking sequences that contain no other confirmed genes (May 2016). Founder line 10 or C9<450B> was identified with ~450 repeats and 3 to 4-fold greater expression of C9orf72 RNA compared to endogenous mouse C9orf72 RNA. |
