B6N.Cg-Tg(C9orf72*)450BLagi/J

Stock number: 029102
Product name: C9^450B
Research areas: Neurobiology Research, Research Tools

Description

Stock No. 029102 has been discontinued. Please see the C9^450B BAC transgenic mouse line (C9B77; Stock No. 030581).

Allele

Symbol: Tg(C9orf72*)450BLagi
Name: transgene insertion 450B, Clotilde Lagier-Tourenne
MGI accession ID: MGI:5776473
Generation method: Transgenic
Attributes: Inserted expressed sequence; Humanized sequence
Synonyms: C9^450B
Marker symbol: Tg(C9orf72*)450BLagi
Marker name: transgene insertion 450B, Clotilde Lagier-Tourenne
Chromosome: UN
Strain of origin: C57BL/6 x C3H
Expressed genes: C9orf72,chromosome 9 open reading frame 72,human
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.
General note: Phenotypic Similarity to Human Syndrome in Orthologous Human Gene: (FRONTOTEMPORAL DEMENTIA AND/OR AMYOTROPHIC LATERAL SCLEROSIS 1; FTDALS1)