Alterations of Amino Acids and Monoamine Metabolism in Male Fmr1 Knockout Mice: A Putative Animal Model of the Human Fragile X Mental Retardation Syndrome
The Fragile X syndrome, a common form of mental retardation in humans, is caused by silencing the fragile X mental retardation (FMR1) geneleading to the absence of the encoded fragile X mental retardation protein 1 (FMRP). We describe morphological and behavioral abnormalities for both affected huma...
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| Format: | Article |
| Language: | English |
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Wiley
2001-01-01
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| Series: | Neural Plasticity |
| Online Access: | http://dx.doi.org/10.1155/NP.2001.285 |
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| author | Michael Gruss Katharina Braun |
| author_facet | Michael Gruss Katharina Braun |
| author_sort | Michael Gruss |
| collection | DOAJ |
| description | The Fragile X syndrome, a common form of
mental retardation in humans, is caused by
silencing the fragile X mental retardation (FMR1)
geneleading to the absence of the encoded fragile
X mental retardation protein 1 (FMRP). We
describe morphological and behavioral abnormalities
for both affected humans and Fmr1
knockout mice, a putative animal model for the
human Fragile X syndrome. The aim of the
present study was to identify possible neurochemical
abnormalities in Fmr1 knockout mice,
with particular focus on neurotransmission.
Significant region-specific differences: of basal
neurotransmitter and metabolite levels were
found between wildtype and Fmr1 knockout
animals, predominantly in juveniles (post-natal
days 28 to 31). Adults (postnatal days 209 to
221) showed only few abnormalities as compared
with the wildtype. In juvenile knockout mice,
aspartate and taurine were especially increased
in cortical regions, striatum, hippocampus, cerebellum,
and brainstem. In addition, juveniles
showed an altered balance between excitatory and
inhibitory amino acids in the caudal cortex,
hippocampus, and brainstem. We detected very
few differences in monoamine turnover in both
age stages. The results presented here provide
the first evidence that lack of FMRP expression
in FMRP knockout mice is accompanied by
age-dependent, region-specific alterations in
neurotransmission. |
| format | Article |
| id | doaj-art-7e2bf01f93aa44bab2ac58b79cf71a27 |
| institution | Kabale University |
| issn | 2090-5904 1687-5443 |
| language | English |
| publishDate | 2001-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Neural Plasticity |
| spelling | doaj-art-7e2bf01f93aa44bab2ac58b79cf71a272025-02-03T01:25:59ZengWileyNeural Plasticity2090-59041687-54432001-01-018428529810.1155/NP.2001.285Alterations of Amino Acids and Monoamine Metabolism in Male Fmr1 Knockout Mice: A Putative Animal Model of the Human Fragile X Mental Retardation SyndromeMichael Gruss0Katharina Braun1Otto von Guericke University Magdeburg, Institute for Biology and Leibniz Institute for Neurobiology, Project Group Juvenile Learning, Brenneckestr. 6, Magdeburg 39118, GermanyOtto von Guericke University Magdeburg, Institute for Biology and Leibniz Institute for Neurobiology, Project Group Juvenile Learning, Brenneckestr. 6, Magdeburg 39118, GermanyThe Fragile X syndrome, a common form of mental retardation in humans, is caused by silencing the fragile X mental retardation (FMR1) geneleading to the absence of the encoded fragile X mental retardation protein 1 (FMRP). We describe morphological and behavioral abnormalities for both affected humans and Fmr1 knockout mice, a putative animal model for the human Fragile X syndrome. The aim of the present study was to identify possible neurochemical abnormalities in Fmr1 knockout mice, with particular focus on neurotransmission. Significant region-specific differences: of basal neurotransmitter and metabolite levels were found between wildtype and Fmr1 knockout animals, predominantly in juveniles (post-natal days 28 to 31). Adults (postnatal days 209 to 221) showed only few abnormalities as compared with the wildtype. In juvenile knockout mice, aspartate and taurine were especially increased in cortical regions, striatum, hippocampus, cerebellum, and brainstem. In addition, juveniles showed an altered balance between excitatory and inhibitory amino acids in the caudal cortex, hippocampus, and brainstem. We detected very few differences in monoamine turnover in both age stages. The results presented here provide the first evidence that lack of FMRP expression in FMRP knockout mice is accompanied by age-dependent, region-specific alterations in neurotransmission.http://dx.doi.org/10.1155/NP.2001.285 |
| spellingShingle | Michael Gruss Katharina Braun Alterations of Amino Acids and Monoamine Metabolism in Male Fmr1 Knockout Mice: A Putative Animal Model of the Human Fragile X Mental Retardation Syndrome Neural Plasticity |
| title | Alterations of Amino Acids and Monoamine Metabolism in
Male Fmr1 Knockout Mice: A Putative Animal Model of
the Human Fragile X Mental Retardation Syndrome |
| title_full | Alterations of Amino Acids and Monoamine Metabolism in
Male Fmr1 Knockout Mice: A Putative Animal Model of
the Human Fragile X Mental Retardation Syndrome |
| title_fullStr | Alterations of Amino Acids and Monoamine Metabolism in
Male Fmr1 Knockout Mice: A Putative Animal Model of
the Human Fragile X Mental Retardation Syndrome |
| title_full_unstemmed | Alterations of Amino Acids and Monoamine Metabolism in
Male Fmr1 Knockout Mice: A Putative Animal Model of
the Human Fragile X Mental Retardation Syndrome |
| title_short | Alterations of Amino Acids and Monoamine Metabolism in
Male Fmr1 Knockout Mice: A Putative Animal Model of
the Human Fragile X Mental Retardation Syndrome |
| title_sort | alterations of amino acids and monoamine metabolism in male fmr1 knockout mice a putative animal model of the human fragile x mental retardation syndrome |
| url | http://dx.doi.org/10.1155/NP.2001.285 |
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