Ontology highlight
ABSTRACT:
ORGANISM(S): Mus musculus
SUBMITTER: Jacqueline O'Rourke
PROVIDER: E-GEOD-77720 | biostudies-arrayexpress |
REPOSITORIES: biostudies-arrayexpress
O'Rourke Jacqueline G JG Bogdanik Laurent L Muhammad A K M G AKMG Gendron Tania F TF Kim Kevin J KJ Austin Andrew A Cady Janet J Liu Elaine Y EY Zarrow Jonah J Grant Sharday S Ho Ritchie R Bell Shaughn S Carmona Sharon S Simpkinson Megan M Lall Deepti D Wu Kathryn K Daughrity Lillian L Dickson Dennis W DW Harms Matthew B MB Petrucelli Leonard L Lee Edward B EB Lutz Cathleen M CM Baloh Robert H RH
Neuron 20151201 5
Noncoding expansions of a hexanucleotide repeat (GGGGCC) in the C9orf72 gene are the most common cause of familial amyotrophic lateral sclerosis and frontotemporal dementia. Here we report transgenic mice carrying a bacterial artificial chromosome (BAC) containing the full human C9orf72 gene with either a normal allele (15 repeats) or disease-associated expansion (∼100-1,000 repeats; C9-BACexp). C9-BACexp mice displayed pathologic features seen in C9orf72 expansion patients, including widespread ...[more]