Maintenance of susceptibility to neurodegeneration following intrastriatal injections of quinolinic acid in a new transgenic mouse model of Huntington's disease.

• A transgenic mouse model of Huntington's disease (R6/1 and R6/2 lines) expressing exon 1 of the HD gene with 115-150 CAG repeats resisted striatal damage following injection of quinolinic acid and other neurotoxins. We examined whether excitotoxin resistance characterizes mice with mutant huntingtin transgenes. In a new transgenic mouse with 3 kb of mutant human huntingtin cDNA with 18, 46, or 100 CAG repeats, we found no change in susceptibility to intrastriatal injections of the excitotoxin quinolinic acid, compared to wild-type littermates. The new transgenic mice were injected with the same dose of quinolinic acid (30 nmol) as had been the R6 mice. Our findings highlight the importance of studying pathogenetic mechanisms in different transgenic models of a disease.

Ämnesord

MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Neurologi (hsv//swe)

MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Neurology (hsv//eng)

Nyckelord

Huntington Disease : complications

Heterozygote

Huntington Disease : chemically induced

Human

Huntington Disease : genetics

P.H.S.

U.S. Gov't

Support

Non-P.H.S.

Non-U.S. Gov't

Species Specificity

Quinolinic Acid : administration & dosage

Neurons : pathology

Neurons : drug effects

Neurodegenerative Diseases : pathology

Neurodegenerative Diseases : complications

Neurodegenerative Diseases : chemically induced

Microinjections

Transgenic

Mice

Huntington Disease : pathology

Disease Susceptibility

Animal

Disease Models

Corpus Striatum : pathology

Cell Count

Cell Death

Corpus Striatum : drug effects

Publikations- och innehållstyp

art (ämneskategori)

ref (ämneskategori)