?url_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Adc&rft.title=A+phenotypic+and+molecular+characterization+of+the+fmr1-tm1Cgr+Fragile+X+mouse&rft.creator=Bauchwitz%2C+Dr.+Robert+P.&rft.subject=Behavioral+Neuroscience&rft.description=Fragile+X+Syndrome+is+the+most+common+form+of%0D%0Ainherited+mental+retardation.+It+is+also+known+for+having%0D%0Aa+substantial+behavioral+morbidity%2C+including+autistic+features.+In+humans%2C+Fragile+X+Syndrome+is+almost+always%0D%0Acaused+by+inactivation+of+the+X-linked+FMR1+gene.+A%0D%0Asingle+knockout+mouse+model%2C+fmr1-tm1Cgr%2C+exists.+In%0D%0Athis+report+we+further+characterize+the+cognitive+and%0D%0Abehavioral+phenotype+of+the+fmr1-tm1Cgr+Fragile+X%0D%0Amouse+through+the+use+of+F1+hybrid+mice+derived+from%0D%0Atwo+inbred+strains+(FVB%2FNJ+and+C57BL%2F6J).+Use+of+F1%0D%0Ahybrids+allows+focus+on+the+effects+of+the+fmr1-tm1Cgr%0D%0Aallele+with+reduced+influence+from+recessive+alleles%0D%0Apresent+in+the+parental+inbred+strains.+We+find+that+the%0D%0Acognitive+phenotype+of+fmr1-tm1Cgr+mice%2C+including%0D%0Ameasures+of+working+memory+and+learning+set+formation%0D%0Athat+are+known+to+be+seriously+impacted+in+humans+with%0D%0AFragile+X+Syndrome%2C+are+essentially+normal.+Further+testing+of+inbred+strains+supports+this+conclusion.+Thus%2C+any%0D%0Afmr1-tm1Cgr+cognitive+deficit+is+surprisingly+mild+or%0D%0Aabsent.+There+is%2C+however%2C+clear+support+presented+for+a%0D%0Arobust+audiogenic+seizure+phenotype+in+all+strains+tested%2C%0D%0Aas+well+as+increased+entries+into+the+center+of+an+open%0D%0Afield.+Finally%2C+a+molecular+examination+of+the+fmr1-tm1Cgr%0D%0Amouse+shows+that%2C+contrary+to+common+belief%2C+it+is+not+a%0D%0Amolecular+null.+Implications+of+this+finding+for+interpretation+of+the+phenotype+are+discussed.%0D%0A&rft.publisher=Blackwell+Munksgaard+&rft.date=2004-05-10&rft.type=Journal+(Paginated)&rft.type=PeerReviewed&rft.format=application%2Fpdf&rft.identifier=http%3A%2F%2Fcogprints.org%2F6539%2F1%2FYan2004_G2B_offprint.pdf&rft.identifier=++Bauchwitz%2C+Dr.+Robert+P.++(2004)+A+phenotypic+and+molecular+characterization+of+the+fmr1-tm1Cgr+Fragile+X+mouse.++%5BJournal+(Paginated)%5D+++++&rft.relation=http%3A%2F%2Fcogprints.org%2F6539%2F