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Written by | My best friend Red Hat
Sr.
's ability to distinguish the acoustic frequencies of each other and the surrounding environment is extremely important
in the survival, development and evolution of species.
We humans also receive information from the outside world through language and music every day [1, 2], and learning musical instruments requires cultivating the ability to distinguish between high frequencies [3].
Conversely, if audio discrimination is low, language ability will also be weakened [4].
However, the neurological and genetic basis of audio discrimination is still poorly
understood.
Williams-Beuren syndrome is a natural model
for studying this problem.
It is a neurodevelopmental disorder caused by mutations in chromosome 7q11.
23 [5].
Although patients with Williams syndrome have developmental delays, mental retardation, and cognitive impairment, they have musical and language talents that are far superior to ordinary people [6].
These patients tend to be interested in specific sounds, especially music
.
The auditory-related neuroanatomy of Williams syndrome patients is also different from ordinary people, and audio discrimination and language and music perception are generally controlled
by the auditory cortex of the brain.
Although the overall cortex volume of Williams syndrome patients is less than normal, the auditory cortex volume is increasing [7].
Moreover, compared with normal people, patients with Williams syndrome have elevated auditory evoked potentials and enhanced abnormal activation of the sound-activated cortex, which indicates that auditory cortical abnormalities may be directly related
to Williams syndrome.
It has been reported that the auditory cortex can regulate audio discrimination [8].
Optogenetic activation of PV+GABAergic interneurons on the auditory cortex can improve behavior to some extent, and this process relies on audio discrimination
.
Recently, St.
The Stanislav S.
Zakharenko research group at Jude Children's Research Hospital published an article in Cell titled Innate frequency-discrimination hyperacuity in Williams-Beuren syndrome mice.
The specific working mechanism
of audio discrimination ability was studied by using a mouse model of Williams syndrome.
First, the authors took 6-12 weeks Williams syndrome model mice as the research object, and found that Williams syndrome mice were better than control wild-type mice
in terms of audio discrimination ability through the detection experiment of auditory startle response based on prepulse suppression.
Moreover, the peripheral hearing of the Williams syndrome mice did not change, indicating that their enhanced audio discrimination ability came from the central hearing system
.
Synaptic interactions in the auditory cortex can affect audio discrimination in mice
.
Subsequently, the authors studied neurons in the auditory cortex and the auditory thalamus and the neural circuits
between them.
The authors found that cortical excitatory neurons in Williams syndrome mice received similar excitatory stimuli compared with normal control mice, but the inhibitory stimuli were significantly enhanced, resulting in reduced
spontaneous excitatory synaptic activity in the auditory cortex.
The reason may be that the activation voltage of the inhibitory interneuron introverted current in the auditory cortex of mice with Williams syndrome is basically consistent with its action potential threshold, resulting in its continuous high activation
.
Moreover, the highly activated inhibitory interneurons described above have been confirmed by the authors to be one of
the main reasons for their enhanced audio discrimination ability.
Next, the authors further investigate the specific mechanism of
the above phenomenon.
The authors found that tone-induced neural responses in the auditory cortex were the ability
of Williams syndrome mice to obtain better encoding of audio information.
The authors also determined by knocking out different genes related to Williams syndrome that the deletion of Gtf2ird1+/- hemizygotes is the cause of the
strong audio discrimination ability of Williams syndrome mice.
Finally, the authors study the transcription factor Gtfird1 and its target and its relationship
with audio discrimination.
The authors found through RNA sequencing and other methods that Gtfird1 deletion can significantly downregulate the level of
Vipr1.
VIPR1 levels are also significantly downregulated
in auditory cortical interneurons in Williams syndrome patients.
VIPR1 antagonists can basically mimic the state of interneurons in the auditory cortex of Williams syndrome mice, which also indicates that the VIPR1 signaling pathway is involved in the high activation of
inhibitory neurons.
For mice with Williams syndrome, inhibition of the auditory cortex interneuron Vipr1 is a necessary factor
for its superior audio discrimination ability.
In summary, the authors have superior audio discrimination ability through a mouse model of Williams syndrome, and this ability is caused
by the downregulation of inhibitory interneuron Vipr1 in the auditory cortex.
Platemaker: Eleven
1.
Peretz, I.
(2016).
Neurobiology of congenital amusia.
Trends Cogn.
Sci.
20,857–867.
https://doi.
org/10.
1016/j.
tics.
2016.
09.
002.
2.
Gervain, J.
, and Geffen, M.
N.
(2019).
Efficient neural coding in auditory and speech perception.
Trends Neurosci.
42, 56–65.
https://doi.
org/10.
1016/j.
tins.
2018.
09.
004.
3.
Micheyl, C.
, Delhommeau, K.
, Perrot, X.
, and Oxenham, A.
J.
(2006).
Influence of musical and psychoacoustical training on pitch discrimination.
Hear.
Res.
219, 36–47.
https://doi.
org/10.
1016/J.
HEARES.
2006.
05.
004.
4.
Kleindienst, L.
, and Musiek, F.
(2011).
Do frequency discrimination deficits lead to specific language impairments? Hear.
J.
64, 10–11.
https://doi.
org/10.
1097/01.
HJ.
0000396582.
67365.
34.
5.
Kozel, B.
, Barak, B.
, Kim, C.
, Mervis, C.
, Osborne, L.
, Porter, M.
, and Pober, B.
(2021).
Williams syndrome.
Nat.
Rev.
Dis.
Prim.
7, 1–22.
https://doi.
org/10.
1038/S41572-021-00276-Z.
6.
Morris, C.
A.
, and Braddock, S.
R.
; COUNCIL ON GENETICS (2020).
Health care supervision for children With Williams syndrome.
Pediatrics 145, e20193761.
https://doi.
org/10.
1542/PEDS.
2019-3761.
7.
Martens, M.
A.
, Reutens, D.
C.
, and Wilson, S.
J.
(2010).
Auditory cortical volumes and musical ability in Williams syndrome.
Neuropsychologia 48, 2602–2609.
https://doi.
org/10.
1016/j.
neuropsychologia.
2010.
05.
007.
8.
Aizenberg, M.
, and Geffen, M.
N.
(2013).
Bidirectional effects of aversive learning on perceptual acuity are mediated by the sensory cortex.
Nat.
Neurosci.
16, 994–996.
https://doi.
org/10.
1038/nn.
3443.
Sr.
's ability to distinguish the acoustic frequencies of each other and the surrounding environment is extremely important
in the survival, development and evolution of species.
We humans also receive information from the outside world through language and music every day [1, 2], and learning musical instruments requires cultivating the ability to distinguish between high frequencies [3].
Conversely, if audio discrimination is low, language ability will also be weakened [4].
However, the neurological and genetic basis of audio discrimination is still poorly
understood.
Williams-Beuren syndrome is a natural model
for studying this problem.
It is a neurodevelopmental disorder caused by mutations in chromosome 7q11.
23 [5].
Although patients with Williams syndrome have developmental delays, mental retardation, and cognitive impairment, they have musical and language talents that are far superior to ordinary people [6].
These patients tend to be interested in specific sounds, especially music
.
The auditory-related neuroanatomy of Williams syndrome patients is also different from ordinary people, and audio discrimination and language and music perception are generally controlled
by the auditory cortex of the brain.
Although the overall cortex volume of Williams syndrome patients is less than normal, the auditory cortex volume is increasing [7].
Moreover, compared with normal people, patients with Williams syndrome have elevated auditory evoked potentials and enhanced abnormal activation of the sound-activated cortex, which indicates that auditory cortical abnormalities may be directly related
to Williams syndrome.
It has been reported that the auditory cortex can regulate audio discrimination [8].
Optogenetic activation of PV+GABAergic interneurons on the auditory cortex can improve behavior to some extent, and this process relies on audio discrimination
.
Recently, St.
The Stanislav S.
Zakharenko research group at Jude Children's Research Hospital published an article in Cell titled Innate frequency-discrimination hyperacuity in Williams-Beuren syndrome mice.
The specific working mechanism
of audio discrimination ability was studied by using a mouse model of Williams syndrome.
First, the authors took 6-12 weeks Williams syndrome model mice as the research object, and found that Williams syndrome mice were better than control wild-type mice
in terms of audio discrimination ability through the detection experiment of auditory startle response based on prepulse suppression.
Moreover, the peripheral hearing of the Williams syndrome mice did not change, indicating that their enhanced audio discrimination ability came from the central hearing system
.
Synaptic interactions in the auditory cortex can affect audio discrimination in mice
.
Subsequently, the authors studied neurons in the auditory cortex and the auditory thalamus and the neural circuits
between them.
The authors found that cortical excitatory neurons in Williams syndrome mice received similar excitatory stimuli compared with normal control mice, but the inhibitory stimuli were significantly enhanced, resulting in reduced
spontaneous excitatory synaptic activity in the auditory cortex.
The reason may be that the activation voltage of the inhibitory interneuron introverted current in the auditory cortex of mice with Williams syndrome is basically consistent with its action potential threshold, resulting in its continuous high activation
.
Moreover, the highly activated inhibitory interneurons described above have been confirmed by the authors to be one of
the main reasons for their enhanced audio discrimination ability.
Next, the authors further investigate the specific mechanism of
the above phenomenon.
The authors found that tone-induced neural responses in the auditory cortex were the ability
of Williams syndrome mice to obtain better encoding of audio information.
The authors also determined by knocking out different genes related to Williams syndrome that the deletion of Gtf2ird1+/- hemizygotes is the cause of the
strong audio discrimination ability of Williams syndrome mice.
Finally, the authors study the transcription factor Gtfird1 and its target and its relationship
with audio discrimination.
The authors found through RNA sequencing and other methods that Gtfird1 deletion can significantly downregulate the level of
Vipr1.
VIPR1 levels are also significantly downregulated
in auditory cortical interneurons in Williams syndrome patients.
VIPR1 antagonists can basically mimic the state of interneurons in the auditory cortex of Williams syndrome mice, which also indicates that the VIPR1 signaling pathway is involved in the high activation of
inhibitory neurons.
For mice with Williams syndrome, inhibition of the auditory cortex interneuron Vipr1 is a necessary factor
for its superior audio discrimination ability.
In summary, the authors have superior audio discrimination ability through a mouse model of Williams syndrome, and this ability is caused
by the downregulation of inhibitory interneuron Vipr1 in the auditory cortex.
Original link:
https://doi.
org/10.
1016/j.
cell.
2022.
08.
022
Platemaker: Eleven
References
1.
Peretz, I.
(2016).
Neurobiology of congenital amusia.
Trends Cogn.
Sci.
20,857–867.
https://doi.
org/10.
1016/j.
tics.
2016.
09.
002.
2.
Gervain, J.
, and Geffen, M.
N.
(2019).
Efficient neural coding in auditory and speech perception.
Trends Neurosci.
42, 56–65.
https://doi.
org/10.
1016/j.
tins.
2018.
09.
004.
3.
Micheyl, C.
, Delhommeau, K.
, Perrot, X.
, and Oxenham, A.
J.
(2006).
Influence of musical and psychoacoustical training on pitch discrimination.
Hear.
Res.
219, 36–47.
https://doi.
org/10.
1016/J.
HEARES.
2006.
05.
004.
4.
Kleindienst, L.
, and Musiek, F.
(2011).
Do frequency discrimination deficits lead to specific language impairments? Hear.
J.
64, 10–11.
https://doi.
org/10.
1097/01.
HJ.
0000396582.
67365.
34.
5.
Kozel, B.
, Barak, B.
, Kim, C.
, Mervis, C.
, Osborne, L.
, Porter, M.
, and Pober, B.
(2021).
Williams syndrome.
Nat.
Rev.
Dis.
Prim.
7, 1–22.
https://doi.
org/10.
1038/S41572-021-00276-Z.
6.
Morris, C.
A.
, and Braddock, S.
R.
; COUNCIL ON GENETICS (2020).
Health care supervision for children With Williams syndrome.
Pediatrics 145, e20193761.
https://doi.
org/10.
1542/PEDS.
2019-3761.
7.
Martens, M.
A.
, Reutens, D.
C.
, and Wilson, S.
J.
(2010).
Auditory cortical volumes and musical ability in Williams syndrome.
Neuropsychologia 48, 2602–2609.
https://doi.
org/10.
1016/j.
neuropsychologia.
2010.
05.
007.
8.
Aizenberg, M.
, and Geffen, M.
N.
(2013).
Bidirectional effects of aversive learning on perceptual acuity are mediated by the sensory cortex.
Nat.
Neurosci.
16, 994–996.
https://doi.
org/10.
1038/nn.
3443.
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