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Correction to: Pathophysiology of and therapeutic options for a GABRA1 variant linked to epileptic encephalopathy.

Yun-Fei Bai1,2, Michelle Chiu3, Elizabeth S Chan1, Peter Axerio-Cilies1, Jie Lu1, Linda Huh3, Mary B Connolly3, Ilaria Guella4, Matthew J Farrer4,5, Zhi-Qing David Xu2, Lidong Liu6, Michelle Demos7, Yu Tian Wang8.   

Abstract

Following publication of the original article [1], the authors reported errors in Figure 4.

Entities:  

Year:  2020        PMID: 32220239      PMCID: PMC7102430          DOI: 10.1186/s13041-020-00593-6

Source DB:  PubMed          Journal:  Mol Brain        ISSN: 1756-6606            Impact factor:   4.041


Correction to: Mol Brain (2019) 12:92 https://doi.org/10.1186/s13041-019-0513-9 Following publication of the original article [1], the authors reported errors in Fig. 4. Specifically, a wrong actin blot is presented in Fig. 4a. In this Correction, the corrected version of Fig. 4 is shown.
Fig. 4

The R214C mutation resulted in reduced surface and total expression levels of the α1 subunit, and altered the kinetic and single channel properties of GABAARs. a Representative blots of biotinylation samples for surface receptor expression and cell lysates for total receptor expression from HEK293 cells expressing either WT or R214C GABAARs. b Quantification of surface α1 subunits normalized to Na+/K+ ATPase (n = 6), and total α1 subunits normalized to β-actin (n = 10). Statistical differences were determined using student’s t-test by comparing to expression levels of WT GABAAR expressing cells (***p < 0.001). c Representative traces of GABA currents recorded in excised macro-patch membrane under outside-out configuration from WT or R214C GABAAR expressing cells. Currents were evoked by rapidly perfusion of 10 mM GABA to the membrane patch for 400 ms. Quantification of averaged peak current amplitudes (d), 10–90% rise time (e), deactivation rate (f) and desensitization (g) in WT (n = 8) or R214C (n = 8) GABAAR expressing cells. h Representative single channel current traces recorded under cell-attached configuration with a pipette containing GABA (1 mM) at a holding potential of + 100 mV from cells expressing WT or R214C GABAARs. Quantified average of conductance (i), opening frequency (j), mean open time (k), total open time (l), total closed time (m), and open channel probability (n) of WT (n = 10) or R214C (n = 13) GABAARs. Statistical differences were determined using student’s t-test by comparing to WT GABAAR cells (*p < 0.05, **p < 0.01, ***p < 0.001)

The R214C mutation resulted in reduced surface and total expression levels of the α1 subunit, and altered the kinetic and single channel properties of GABAARs. a Representative blots of biotinylation samples for surface receptor expression and cell lysates for total receptor expression from HEK293 cells expressing either WT or R214C GABAARs. b Quantification of surface α1 subunits normalized to Na+/K+ ATPase (n = 6), and total α1 subunits normalized to β-actin (n = 10). Statistical differences were determined using student’s t-test by comparing to expression levels of WT GABAAR expressing cells (***p < 0.001). c Representative traces of GABA currents recorded in excised macro-patch membrane under outside-out configuration from WT or R214C GABAAR expressing cells. Currents were evoked by rapidly perfusion of 10 mM GABA to the membrane patch for 400 ms. Quantification of averaged peak current amplitudes (d), 10–90% rise time (e), deactivation rate (f) and desensitization (g) in WT (n = 8) or R214C (n = 8) GABAAR expressing cells. h Representative single channel current traces recorded under cell-attached configuration with a pipette containing GABA (1 mM) at a holding potential of + 100 mV from cells expressing WT or R214C GABAARs. Quantified average of conductance (i), opening frequency (j), mean open time (k), total open time (l), total closed time (m), and open channel probability (n) of WT (n = 10) or R214C (n = 13) GABAARs. Statistical differences were determined using student’s t-test by comparing to WT GABAAR cells (*p < 0.05, **p < 0.01, ***p < 0.001)
  1 in total

1.  Pathophysiology of and therapeutic options for a GABRA1 variant linked to epileptic encephalopathy.

Authors:  Yun-Fei Bai; Michelle Chiu; Elizabeth S Chan; Peter Axerio-Cilies; Jie Lu; Linda Huh; Mary B Connolly; Ilaria Guella; Matthew J Farrer; Zhi-Qing David Xu; Lidong Liu; Michelle Demos; Yu Tian Wang
Journal:  Mol Brain       Date:  2019-11-10       Impact factor: 4.041

  1 in total

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