Cerebral Cation Shifts and Amino Acids in Huntington's Disease

Early white matter abnormalities, progressive brain pathology and motor deficits in a novel knock-in mouse model of Huntington's disease

“…These findings indicate that altered brain metabolite levels may serve as alternative biomarkers in clinical trials. We found that significant altered striatal metabolites were detected in 9-and 12-month-old HdhQ250 mice, these changes are reminiscent of some changes in human HD brain (69,70), such as decreased NAA and Glu levels and increased Taurine. The disturbed metabolites are not simply due to neuronal loss, as some metabolites (NAA, Glu) decreased, and the other (Taurine) increased in HD brain.…”

Early white matter abnormalities, progressive brain pathology and motor deficits in a novel knock-in mouse model of Huntington's disease

“…These findings indicate that altered brain metabolite levels may serve as alternative biomarkers in clinical trials. We found that significant altered striatal metabolites were detected in 9-and 12-month-old HdhQ250 mice, these changes are reminiscent of some changes in human HD brain (69,70), such as decreased NAA and Glu levels and increased Taurine. The disturbed metabolites are not simply due to neuronal loss, as some metabolites (NAA, Glu) decreased, and the other (Taurine) increased in HD brain.…”

Characterization of Behavioral, Neuropathological, Brain Metabolic and Key Molecular Changes in zQ175 Knock-In Mouse Model of Huntington’s Disease

“…Other studies also suggested disturbed brain metabolites in HD patients [ 22 – 24 ] and HD mice [ 25 , 26 ], indicating that altered brain metabolite levels may serve as alternative biomarkers in clinical trials. We detected significantly altered striatal metabolites in 6, 9, and 12 months old zQ175 mice, reminiscent of some changes in human HD brain [ 27 , 28 ], such as decreased NAA and GLU levels and increased taurine. The disturbed metabolites are not simply due to neuronal loss, as some metabolites (NAA, GLU) decreased, and others (taurine) increased in HD brain.…”

Creatine supplementation lowers brain glutamate levels in Huntington?s disease

“…Our own preliminary data show as a trend (p = 0.058) elevated Glx concentrations in HD as compared with age-matched controls (data not shown). Some post mortem studies, however, show diminished Glu and normal Gln in HD basal ganglia [21,40,41].…”

Early white matter abnormalities, progressive brain pathology and motor deficits in a novel knock-in mouse model of Huntington's disease

“…These findings indicate that altered brain metabolite levels may serve as alternative biomarkers in clinical trials. We found that significant altered striatal metabolites were detected in 9-and 12-month-old HdhQ250 mice, these changes are reminiscent of some changes in human HD brain (69,70), such as decreased NAA and Glu levels and increased Taurine. The disturbed metabolites are not simply due to neuronal loss, as some metabolites (NAA, Glu) decreased, and the other (Taurine) increased in HD brain.…”

Characterization of Behavioral, Neuropathological, Brain Metabolic and Key Molecular Changes in zQ175 Knock-In Mouse Model of Huntington’s Disease

“…Other studies also suggested disturbed brain metabolites in HD patients [ 22 – 24 ] and HD mice [ 25 , 26 ], indicating that altered brain metabolite levels may serve as alternative biomarkers in clinical trials. We detected significantly altered striatal metabolites in 6, 9, and 12 months old zQ175 mice, reminiscent of some changes in human HD brain [ 27 , 28 ], such as decreased NAA and GLU levels and increased taurine. The disturbed metabolites are not simply due to neuronal loss, as some metabolites (NAA, GLU) decreased, and others (taurine) increased in HD brain.…”

Creatine supplementation lowers brain glutamate levels in Huntington?s disease

“…Our own preliminary data show as a trend (p = 0.058) elevated Glx concentrations in HD as compared with age-matched controls (data not shown). Some post mortem studies, however, show diminished Glu and normal Gln in HD basal ganglia [21,40,41].…”

Early white matter abnormalities, progressive brain pathology and motor deficits in a novel knock-in mouse model of Huntington's disease

“…These findings indicate that altered brain metabolite levels may serve as alternative biomarkers in clinical trials. We found that significant altered striatal metabolites were detected in 9-and 12-month-old HdhQ250 mice, these changes are reminiscent of some changes in human HD brain (69,70), such as decreased NAA and Glu levels and increased Taurine. The disturbed metabolites are not simply due to neuronal loss, as some metabolites (NAA, Glu) decreased, and the other (Taurine) increased in HD brain.…”

Characterization of Behavioral, Neuropathological, Brain Metabolic and Key Molecular Changes in zQ175 Knock-In Mouse Model of Huntington’s Disease

“…Other studies also suggested disturbed brain metabolites in HD patients [ 22 – 24 ] and HD mice [ 25 , 26 ], indicating that altered brain metabolite levels may serve as alternative biomarkers in clinical trials. We detected significantly altered striatal metabolites in 6, 9, and 12 months old zQ175 mice, reminiscent of some changes in human HD brain [ 27 , 28 ], such as decreased NAA and GLU levels and increased taurine. The disturbed metabolites are not simply due to neuronal loss, as some metabolites (NAA, GLU) decreased, and others (taurine) increased in HD brain.…”

Creatine supplementation lowers brain glutamate levels in Huntington?s disease

“…Our own preliminary data show as a trend (p = 0.058) elevated Glx concentrations in HD as compared with age-matched controls (data not shown). Some post mortem studies, however, show diminished Glu and normal Gln in HD basal ganglia [21,40,41].…”