Cysteine string protein and its role in neurodegenerative diseases

A method of making a transgenic mouse whose cells whose cells express a reduced level of CSP as compared to the level of CSP protein expressed in a non-transgenic mouse of the same strain comprising:

• (a) introducing a DNA fragment comprising an altered CSP nucleic acid that encodes a biologically inactive CSP protein or expressing reduced levels of CSP protein as compared to wild-type levels of expression into embryonic stem cells under conditions whereby said DNA fragment becomes integrated into the genome of the embryonic stem cells;
• (b) transferring said embryonic stem cells into blastocysts;
• (c) implanting said blastocysts into the reproductive tract of a pseudopregnant female recipient host mouse;
• and
• (d) maintaining said female recipient host mouse under conditions whereby transgenic offspring are produced.

A method of making a transgenic mouse whose cells whose cells overexpress CSP protein as compared to the level of CSP protein expressed in a non-transgenic animal of the same strain comprising:

• (a) introducing a DNA fragment comprising a full-length CSP open reading frame operably linked to a promoter and a polyadenylation signal into embryonic stem cells under conditions whereby said DNA fragment becomes integrated into the genome of the embryonic stem cells;
• (b) transferring said embryonic stem cells into blastocysts;
• (c) implanting said blastocysts into the reproductive tract of a pseudopregnant female recipient host mouse;
• and
• (d) maintaining said female recipient host mouse under conditions whereby a transgenic mouse offspring is produced.