Mouse model for Huntington's Disease and related DNA sequences

A transgenic mouse having a CAG trinucleotide repeat expansion in excess of 35 commencing at about nucleotide 169 in the translated coding region of the murine HD gene, said transgenic mouse exhibiting cognitive or motor dysfunction demonstrable by comparing said transgenic mouse to a normal control mouse in at least one test selected from the group consisting of motor activity testing, the T-maze alteration test, the radial-arm test and the Morris water maze task.

A targeting vector for introducing a heterologous nucleotide sequence into exon five of DNA of SEQ ID NO:7, whereby transcription of said exon is disrupted.

A transgenic mouse whose diploid cells contain one nucleotide sequence of SEQ ID NO:7 that has been modified by introduction of heterologous DNA within exon five of said nucleotide sequence, whereby transcription of said nucleotide sequence is disrupted so that a full-length protein product is not produced, said transgenic mouse exhibiting cognitive or motor dysfunction demonstrable by comparing said transgenic mouse to a normal control mouse in at least one test selected from the group consisting of motor activity testing, the T-maze alteration test, the radial-arm test and the Morris water maze task.

A transgenic mouse whose diploid cells contain, within exon five of one allele of the murine HD gene, a heterologous DNA sequence that disrupts transcription of the murine HD gene, so that a full-length murine HD protein product is not produced, and wherein said transgenic mouse exhibits cognitive or motor dysfunction demonstrable by comparing said transgenic mouse to a normal control mouse in at least one test selected from the group consisting of motor activity testing, the T-maze alteration test, the radial-arm maze test and the Morris water maze task.

A transgenic mouse having a trinucleotide CAG repeat expansion in excess of 35 commencing at about nucleotide 169 of the translated coding region of the murine HD gene, wherein said transgenic mouse exhibits cognitive or motor dysfunction demonstrable by comparing said transgenic mouse to a normal control mouse in at least one test selected from the group consisting of motor activity testing, the T-maze alteration test, the radial-arm maze test and the Morris water maze task.

A transgenic mouse whose cells contain a nucleotide sequence encoding a human huntingtin protein, said nucleotide sequence comprising a region containing more than 35 CAG trinucleotide repeats, wherein said transgenic mouse exhibits cognitive or motor dysfunction demonstrable by comparing said transgenic mouse to a normal control mouse in at least one test selected from the group consisting of motor activity testing, the T-maze alteration test, the radial-arm maze test and the Morris water maze task.

An oligonucleotide probe consisting of at least 18 contiguous nucleic acid residues selected from SEQ ID NO:7.

An isolated DNA molecule having SEQ ID NO:7.

A cloning vector comprising DNA of SEQ ID NO:7, wherein said DNA molecule is operatively linked to an expression control sequence.