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FUSDelta14 Knock-in

RESEARCH MODELS Summary The FUSDelta14 mutation (g.13845A>G) at the splice acceptor site of intron 13 results in skipping of exon 14, out-of-frame translation of exon 15, and C-terminal truncation of the FUS protein (FUS p.G466VfsX14). This mutation removes the ...

E2FAD

RESEARCH MODELS The E4FAD, E3FAD, and E2FAD mouse models are crosses between the widely used 5xFAD mice (Tg6799 line) and the APOE4, APOE3, and APOE2 Targeted Replacement mice, respectively. These models were created to study the role of the three human isoforms of APOE ...

E3FAD

RESEARCH MODELS The E4FAD, E3FAD, and E2FAD mouse models are crosses between the widely used 5xFAD mice (Tg6799 line) and the APOE4, APOE3, and APOE2 Targeted Replacement mice, respectively. These models were created to study the role of the three human isoforms of APOE ...

E4FAD

RESEARCH MODELS The E4FAD, E3FAD, and E2FAD mouse models are crosses between the widely used 5xFAD mice (Tg6799 line) and the APOE4, APOE3, and APOE2 Targeted Replacement mice, respectively. These models were created to study the role of the three human isoforms of APOE ...

FusΔNLS

RESEARCH MODELS Summary FUS (Fused in Sarcoma) is a member of a family of RNA-binding proteins with roles in transcription, RNA processing, RNA transport, and translation. Mutations in FUS, many of which are clustered near the nuclear localization signal (NLS) at the C ...

ΔNLS-FUS x TDP-43(WT)

RESEARCH MODELS This mouse model of ALS is a cross between the  ΔNLS-FUS line, which overexpresses human FUS lacking the nuclear localization signal (NLS), and the TDP-43(WT) line, which overexpresses wild-type human TDP-43 (Shiihashi et al., 2016). Both lines utilize a ...

ΔNLS-FUS

RESEARCH MODELS FUS (Fused in Sarcoma) is a member of a family of RNA-binding proteins with roles in transcription, RNA processing, RNA transport, and translation. Mutations in FUS, many of which are clustered near the nuclear localization signal (NLS) at the C-terminus, ...

hFUS-R521C

RESEARCH MODELS Summary FUS (Fused in Sarcoma) is a member of a family of RNA-binding proteins with roles in transcription, RNA processing, RNA transport, and translation. Mutations in FUS, including R521C, are linked to rare familial cases of ALS. A transgene containing ...

hFUS-P525L

RESEARCH MODELS Summary FUS (Fused in Sarcoma) is a member of a family of RNA-binding proteins with roles in transcription, RNA processing, RNA transport, and translation. Mutations in FUS, many of which are clustered near the nuclear localization signal at the C ...

LRRK2 G2019S Mouse (Tg)

RESEARCH MODELS Summary These transgenic mice overexpress mutant human LRRK2 in the brain. The mutant LRKK2 protein, with the G2019S mutation, is observed throughout the brain, including the striatum, cerebral cortex, hippocampus, cerebellum, and brainstem (Ramonet et al ...

Parkin Q311X Mouse (BAC Tg)

RESEARCH MODELS Summary This transgenic model of Parkinson’s disease expresses near-physiological levels of mutant parkin in dopaminergic neurons. The Q311X mutation is a nonsense mutation, which produces C-terminally truncated parkin, just 155 amino acids long. Notably, ...

PINK1 G309D (PINK1-/-) Mouse (KI)

RESEARCH MODELS Summary This mouse model is referred to as Pink1-/-. It is not a genetic knockout, but carries an incorrectly spliced and unstable Pink1 mRNA resulting in deficiency of PINK1 protein. This model was initially designed to carry a knock-in mutation (PINK1 ...

LRRK2 WT Mouse (BAC Tg)

RESEARCH MODELS Summary These transgenic mice overexpress human wild-type LRRK2 (Li et al., 2009). The mice develop normally and do not exhibit any known motor deficits. The WT-OX model uses a bacterial artificial chromosome (BAC) to overexpress human LRRK2. The BAC ...

LRRK2 G2019S Mouse (BAC Tg)

RESEARCH MODELS Summary This transgenic mouse overexpresses a mutant form of Lrrk2 in the brain using a bacterial artificial chromosome (BAC) (Li et al., 2010). Transgene expression is driven by the mouse Lrrk2 promoter sequence. Hemizygous mice develop an age-associated ...

PINK1 KO Mouse

RESEARCH MODELS Summary This mouse model was developed to investigate the effects of PINK1 deficiency (Kitada et al., 2007). The model involves a germline deletion of exons 4-7 of the endogenous PINK1 gene, creating truncated transcripts that are degraded. Mice ...

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