EMBO/soda-nel huggingface dataset:
Human cerebral organoids reveal progenitor pathology in EML1-linked cortical malformation (2022)
Figure 1

Figure 1: Deficiency of EML1 causes ectopic neural rosettes and neuronal heterotopia in cerebral organoids.

(A) Day 33±2 control- and EML1-patient-derived cerebral organoids stained for ßIII-Tubulin and DAPI. VZ and CP areas are marked by white brackets, ectopic neural rosettes by red bracket and heterotopic neurons by red arrows.

(B) day 33±2 control and EML1-patient-derived cerebral organoid stained for the adherens junction marker N-cadherin (NCAD) expressed at the VZ surface and within the ectopic neural rosettes.

(C) day 20±2 cerebral organoids derived from controls, patients and EML1-heKOs stained for the neuronal marker MAPT and the adherens junction marker NCAD, counterstained with DAPI. Ectopic neural rosettes and neuronal heterotopia are highlighted with dotted yellow lines.

(D) Quantification of VZ areas with ectopic neural rosettes (3 batches, 3 organoids analyzed per batch, significance based on Kruskal-Wallis-test, P=0.0001, Dunn's post hoc test for multiple comparisons performed to define statistical differences between genotypes, single data points are presented colored by batch).

(E) Quantification of heterotopic, disorganized or organized cortical areas (3 batches, 3 organoids per batch, significance based on Kruskal-Wallis-test, P=0.0001 for "organized" and "heterotopic"; no significant difference for "disorganized", Dunn's post hoc test performed for multiple comparisons to define statistical differences between genotypes).

Data information: * marks statistical significance in relation to Control 1, # in relation to Control 2. P-values: ***/###<0.001. VZ: ventricular zone; CP: cortical plate. Data in graphs are represented as means ± SD. Scale bars: (A) 100µm; (B) 100µm, enlarged 25µm; (C) 50µm.
Human cerebral organoids reveal progenitor pathology in EML1-linked cortical malformation (2022)
Figure 2

Figure 2: Ectopic progenitor cells originate from apical RG with perturbed cell-intrinsic behavior.

(A) Hybrid organoids generated from controls mixed with control-EGFP+ or EML1-heKO-EGFP+ cells (day 20±2). Yellow dotted lines indicate VZ areas.

(B) Quantification of control-EGFP+ or EML1-heKO-EGFP+ cells within or at the basal side of VZ areas (3 batches, 3 organoids each, significance based on Mann Whitney test, ***P= 0.0001 for "EGFP+ cells at the basal side of VZ areas").

(C) Representative images and scheme of cleavage plan orientation. Upper panel: cells immunostained for pVim (red) and TPX2 (green), counterstained with DAPI, white dotted line indicates cleavage plane orientation, yellow dotted line VZ-surface.

(D) Quantification of horizontal, vertical and oblique plane of cell divisions in control and EML1-deficient organoids (day 20±2; 3 batches, 3 organoids each).

(E) Quantification of primary cilia length in control and EML1-deficient cortical progenitors (* marks statistical significance in relation to Control 1, # in relation to Control 2, 3 biological replicates, 4 areas per sample, significance based on Kruskal-Wallis-test and Dunn's post hoc test, ***/###P= 0.0001).

(F) Electron microscopy of primary cilia derived from control or EML1-deficient cortical progenitors in DMSO-control or following EpothiloneD (EpoD) exposure (blue arrow: basal body, yellow arrow: appendages, red arrow: ciliary pockets).

(G) Quantification of primary cilia length in control and EML1-deficient cortical progenitors with/without EpoD exposure. 3 biological replicates, 4 areas per sample, significance based on two-way ANOVA and Sidak's multiple comparisons post hoc test, P=0.0213.

Data information: Data in graphs are represented as means ± SD. Scheme in C was designed using biorender.com. Scale bars: (A) 50µm; (C) 5µm; (F) 0,5µm.
Human cerebral organoids reveal progenitor pathology in EML1-linked cortical malformation (2022)
Figure 3

Figure 3: ScRNA-seq reveals cellular identity of ectopic cells in EML1-heKO cerebral organoids.

(A) scRNA-seq of 2,335 control and 3,358 EML1-heKO cells (3 pooled independent batches, 3 organoids each). Cells shown in UMAP plot and colored by annotated cell type.

(B) Comparison of cell-type composition between control and EML1-heKO. Numbers indicate percentage of total cells belonging to the respective cell-type.

(C) Normalized expression values of COL1A2, COL3A1, LUM and MEIS2 per cell-type (3 pooled batches, 3 organoids each).

(D), Gene ontology (GO) term analysis of RG to bRG cells in control and EML1-heKO shows percentage of counts belonging to the set of genes associated with the respective GO term (3 pooled batches, 3 organoids each).

(E) Control and EML1-heKO-derived cerebral organoid stained for COL1A2 and MEIS2. VZ areas are encircled, squares indicate areas enlarged in F.

(F) Control and EML1-heKO-derived cerebral organoid stained for COL1A2, MEIS2 and MAPT.

(G) Representative images showing EGFP-control and EGFP-EML1-heKO hybrid cerebral organoid stained for EGFP, FAM107A and NCAD. Yellow dotted lines indicate morphology of EGFP+ cells (4 independent batches each, 3 organoids per batch, at least 30 ectopic rosettes in total).

Data information: RG1: radial glia1, RG to bRG: radial glia to basal radial glia, RG2: radial glia to young neurons, IP: intermediate progenitors, YN: young neurons, MLC: mesenchymal-like cells. C and D: Asterisks indicate Bonferroni corrected p-values. Wilcoxon Rank Sum test: ***<0.001, *<0.05, ns: not significant. Scale bars: (E) 50µm; (F) 10µm; (G) 50µm, enlarged 10µm.
Human cerebral organoids reveal progenitor pathology in EML1-linked cortical malformation (2022)
Figure 4

Figure 4: YAP signalling drives ectopic progenitor cell expansion in EML1-deficient organoids.

(A) Quantification of mitotic cells located at the basal side of VZ areas in control and EML1-deficient conditions (3 batches, 3 organoids analyzed per batch).

(B) Quantification of BrdU+ KI67+ cells located at the basal side of VZ areas in control and EML1-deficient conditions (3 batches, 3 organoids analyzed per batch).

(C) Normalized expression of YAP1, TEAD2, CCND3, and CYR61 in RG to bRG cells. Separate violins show expression in control and EML1-heKO. Asterisks indicate Bonferroni corrected p-values. Percentages indicate amount of cells in each group expressing the respective gene (3 pooled batches, 3 organoids each).

(D, E) Immunofluorescence staining for (D) p-VIM and YAP1 or (E) Pax6 and YAP1 in control and EML1-heKO-derived organoids (day 20±2, squares indicate areas enlarged in adjacent part of the panel respectively).

(F) Quantification of basally located pVim+ cells with nuclear YAP1 signal per VZ area in control and EML1-heKO-derived organoids (3 batches, 3 organoids analyzed per batch).

(G) Quantification of PAX6+ cells with nuclear YAP1 signal per VZ area in control and EML1-heKO-derived organoids (3 batches, 3 organoids analyzed per batch).

(H) Immunofluorescence staining for p-Vim in EML1-heKO and control derived organoids in DMSO, Verteporfin or Fluvastatin treated conditions, counterstained with DAPI. Dotted line indicates VZ areas.

(I) Quantification of p-VIM+ cells located at the basal side of VZ areas in control or EML1-heKO-derived organoids under DMSO, Verteporfin or Fluvastatin treated condition (3 batches, 3 organoids analyzed per batch).

(J) Quantification of ectopic neural rosettes per VZ area in EML1-heKO-derived organoids in DMSO, Verteporfin or Fluvastatin treated conditions (3 batches, 3 organoids analyzed per batch).

Data information: Data in graphs are represented as means ± SD. Significance based on Kruskal-Wallis-test (A-B, F, G, J), or two-way ANOVA (on log10 normalized data) (I) or Wilcoxon Rank Sum test (C). Dunn's post hoc (A, F-G, J) or Tukey post hoc (I) test for multiple comparisons were performed to define statistical differences between genotypes. P-values: ***<0.001, **<0.01, *<0.05. Scale bars: (D and E) 50µm, enlarged 10µm; (H) 50µm

Expanded View Figure legends
A Sizer model for cell differentiation in Arabidopsis thaliana root growth (2017)
Figure 5

Figure 5. BRI1 signaling at the dividing cells restores overall root growth

A. Eight-day-old seedlings of WT, bri1 and pRPS5a:BRI1:YFP;bri1. Scale bar 1 cm. B. Root-length measurements of 10-day-old bri1-116, bes1-D and pRP5SA:BRI:YFP;bri1-116 seedlings compared to the wild type. Symbols represent the mean of more than 20 plants, from 3 independent experiments. Bars show standard deviation. Straight lines represent the linear regression applied to each curve starting with day 4 post germination. In this way the root growth rate can be extracted for each phenotype (See Table EV7). C. Confocal images of 8-day-old wild type WT, bri1-116 and pRP5SA:BRI:YFP;bri1-116 roots stained with PI. Green lines label the end of the transition zone and the yellow lines label the first root hair (end of elongation zone). The inset shows pRP5SA expression domain of an 8-day-old pRP5SA:BRI:YFP;bri1-116 seedling. Green line labels the end of the meristematic zone, which coincides with the end of pRP5SA expression domain. Scale bars correspond to 100 microns. C-H. Main relationships as in Fig. 2C-E between experimental values of the phenotypic traits used to assess the mechanism of differentiation in bri1-116 mutant (C,E,G) and pRP5SA:BRI:YFP;bri1-116 line (D,F,H) for the epidermis (left large plot) and cortex (right inset plot). Each circle denotes data extracted from a single root (n=126 (epidermis) and n=25 (cortex) for bri1-116 and n=90 (epidermis) and n=17 (cortex) for pRP5SA:BRI:YFP;bri1-116, see Table EV1, pooled from 6, 8 and 10 day-old seedlings for epidermis and from day 6 for cortex (data in Table EV5)). In G, H, the slope of the linear regression is 2.13±0.04 for bri1-116 and 2.53±0.04 for pRP5SA:BRI:YFP;bri1-116. Pearson correlation coefficient r is indicated and p-value (p, using standard Person correlation test). In E, bri1-116 epidermal data do not conform to normal distributions.
Dysregulation of cystathionine γ-lyase promotes prostate cancer progression and metastasis (2019)
Figure 1

Figure 1. Expression of CTH increases in bone-metastatic PC3 cells and positively correlates with PC progression and poor survival.

(A) Comparison of migration and invasion abilities between T- and B- lines. PC3 T- and B-line cells were incubated for migration (16h) and invasion (24h) assay. Data shown represent the means ± SD (n=3 biological replicates). Student's t-test was used for the statistical analysis (*P<0.05; **P<0.01).

(B) Comparison of cell proliferation rate between T- and B- lines. Cell proliferation assay was performed by MTS reagents. Data are the means ± SD (n=3 biological replicates).

(C) Western blot analysis of the endogenous CTH in PC3 orthotopically implanted primary tumor-derived (T2 and T3-lines) and bone-metastases-derived cancer cells (B2 and B3-lines).

(D) Top: H2S catalyzing activity of cell lysates from PC3 T2, B2, T3, and B3 lines. Lead acetate-soaked paper strips show a PbS brown stain as a result of reaction with H2S. Bottom: The level of H2S production was quantified by densitometry, and the histograms represent the means ± SD (n=3 biological replicates). ANOVA followed by Tukey's post-hoc test was used for the statistical analysis (*P<0.05).

(E, F) 1x106 PC3-T2, B2, T3, and B3 cells were orthotopically injected into mouse prostate for 15-20 days. Incidences of paraaortic lymph nodes metastasis (E) and bone metastasis (F) are shown (n=6-8 mice per group). Student's t-test was used for the statistical analysis (*P<0.05; **P<0.01).

(G) Top: expression of CTH in prostate tumors by immunocytochemistry analysis of commercial tissue arrays. Samples were sub-grouped by TNM stages. The representative images from different stages are shown. S: Stroma; Scale bars: 250μm. Bottom: The statistical significance was determined using the χ2 test

(H, I) The RNA-seq data of CTH was downloaded from the TCGA database as described in the section of materials and methods. (H) The mRNA expression of CTH in PC specimens and normal tissues. Data are presented as means ± SD (n=50 cases for adjancent non-tumor parts and 469 cases for PC tumors). Student's t-test was used for the statistical analysis (**P<0.01). (I) Kaplan-Meier survival analysis of PC patients with high or low CTH expression. The statistical significance was determined using the χ2 test.
Dysregulation of cystathionine γ-lyase promotes prostate cancer progression and metastasis (2019)
Figure 2

Figure 2. CTH promotes H2S production, migration, and invasion.

(A) Western blot analysis of the CTH level in PC3-T2 cells with CTH overexpression by pCMV-CTH-HA or PC3-B2 cells with CTH knockdown by siCTH-1.

(B) Top: The H2S production capacity of cell lysates from PC3-T2 cells with CTH overexpression by pCMV-CTH-HA or PC3-B2 cells with CTH knockdown by siCTH-1. Lead acetate-soaked paper strips show a PbS brown stain as a result of reaction with H2S. Bottom: The level of H2S production was quantified by densitometry, and the histograms represent the means ± SD (n=3 biological replicates). ANOVA followed by Tukey's post-hoc test was used for the statistical analysis (*P<0.05).

(C) PC3-B2 cells were transfected with control or siCTH-1 and then were subjected to migration (16h) and invasion (24h) assays.

(D) Cell proliferation rates of PC3-B2 cells with CTH knockdown. Cell proliferation assay was performed by MTS reagent.

(E) Western blot analysis of the CTH levels in PC3 cells transfected with HA-CTH or pCMV-HA vector control.

(F) PC3 cells were transfected with HA-CTH or pCMV-HA vector control and then incubated for migration (16h) and invasion assay (24h).

(G) PC3 cells were pre-treated with 10μM PAG for 16 hrs, and incubated for migration (16h) and invasion assay (24h) in the presence of 10μM PAG.

Data information: (C, D, F, and G) Data shown represent the means ± SD (n=3 biological replicates). Student's t-test was used for the statistical analysis (*P<0.05; **P<0.01; ***P<0.001).
Dysregulation of cystathionine γ-lyase promotes prostate cancer progression and metastasis (2019)
Figure 3

Figure 3. CTH regulates cell invasion through IL-1β/NF-κB pathway.

(A) Real-Time RT-PCR analysis for IL-1β mRNA levels in PC3 cells with control or CTH knockdown. Recombinant IL-1β (10ng/ml) was added for 24h in serum-free medium.

(B) ELISA assay for IL-1β protein level in the culture medium of PC3 cells with control or CTH knockdown.

(C) PC3 cells transfected with control or CTH knockdown were incubated for invasion (24h) assay with recombinant IL-1β (10ng/ml) added to the medium of the lower chamber.

(D) PC3 cells transfected with control or CTH siRNA were exposed to IL-1β (20ng/ml) for 1hr. Subcellular localization of p65 was detected by immunocytochemistry. Nuclei were counterstained with DAPI. The representative images are shown. Scale bars: 25μm.

(E) The percent of nuclear translocation for p65 was scored by counting the number of nuclear positive stained cells of p65 to the total number of cells in random microscopic fields.

(F, G) Real-Time RT-PCR analysis for VEGF (F) and MMP-13 (G) mRNA level in PC3 cells with control or CTH knockdown. Recombinant IL-1β (10ng/ml) was added for 24h in serum-free medium.

Data information: All data shown (A-C and E-G) represent the means ± SD (n=3 biological replicates). Student's t-test (B) or ANOVA followed by Tukey's post-hoc test (A, C, and E-G) was used for the statistical analysis. (*P<0.05; **P<0.01; ***P<0.001).
Dysregulation of cystathionine γ-lyase promotes prostate cancer progression and metastasis (2019)
Figure 4

Figure 4. H2S promotes cell invasion through IL-1β/NF-κB pathway.

(A) PC3 cells were incubated for migration (16h) and invasion (24h) assay. DMEM/10% FBS, together with various concentration of NaHS (10nM-1mM) acting as a chemoattractant.

(B) PC3 cells transfected with control or CTH siRNA were incubated for invasion (24h) assay. DMEM/10% FBS, with or without NaHS (1μM), acting as a chemoattractant.

(C) Top: PC3 cells treated with 100 μM NaHS for 30 min at 37°C. Cells were lyzed and treated with or without DTT for 10min at 4°C before subjecting to the modified biotin switch assay with the antibody against p65 to detect S-sulfhydration. Down: quantitative analysis of SSH-p65 protein level, and normalized with total p65 level.

(D) PC3 cells were incubated for invasion assay for 24 hrs. DMEM/10% FBS, together with 1μM NaHS and an NF-κB inhibitor (50μg/ml SN50, or 100nM QNZ), acting as a chemoattractant.

(E) Real-Time RT-PCR analysis for IL-1β, VEGF, and MMP-13 mRNA levels in PC3 cells treated with 1μM NaHS for 24h.

(F) ELISA assay for IL-1β protein level in the culture medium of PC3 cells with various concentrations of NaHS (10nM-10μM) added for 24h.

(G) Western blot analysis of the p65 expression in PC3 cells with p65 knock-out and then transfected with p65 wild-type or C38S mutant.

(H) PC3 cells with p65 knock-out with or without overexpression of p65 wild-type or C38S mutant were incubated for invasion assay for 24 hrs. DMEM/10% FBS was used as a chemoattractant.

(I-K) 1x106 PC3 cells with p65 knock-out were transiently re-expressed p65 wild-type or C38S mutant and inoculated into the bloodstream via tail-vein injection. After 7 days of inoculation, mouse lung was collected to examine lung metastasis. (I) Images show H&E staining of the lungs from mice injected with p65 knockout PC3 cells re-expressing p65 wild-type or C38S mutant. Red arrows indicate metastatic nodules. Scale bar: 1mm. (J, K) The total metastatic nodules per lung (J) and the percentage of the metastatic area per lung (K) were quantified. Data are presented as means ± SEM (n=7 mice per group). Student's t-test was used for the statistical analysis (*P<0.05).

Data information: (A-H) Data shown represent the means ± SD (n=3 biological replicates). Student's t-test (E) or ANOVA followed by Tukey's post-hoc test (A, B, C, D, F, and H) was used for the statistical analysis (*P<0.05; **P<0.01; ***P<0.001).
Dysregulation of cystathionine γ-lyase promotes prostate cancer progression and metastasis (2019)
Figure 5

Figure 5. Knockdown of CTH suppresses tumor growth and distant metastasis in the orthotopic implantation mouse model.

(A) Western blot analysis of the CTH expression in PC3 cells stably expressing control or CTH shRNA.

(B-E) 1x106 PC3 cells with control or CTH knockdown were orthotopically injected into the mouse prostate for 60 days. The tumor weight (B), and tumor volume (C) were compared. Incidences of paraaortic lymph nodes metastasis (D) and bone metastasis (E) are shown. Data are presented as means ± SEM (n = 10 mice per group).

(F) Real-Time RT-PCR analysis for CTH mRNA levels in PC3 orthotopic tumors from shCon or shCTH group. Data shown represent normalized means ± SEM (n=10 mice per group).

(G, H) Immunohistochemical analysis of paraffin-embedded xenograft prostate tumor using CD31 (G) and LYVE-1 (H) antibody. The total vessels number per mm2 was quantified. Scale bars: 0.1 mm. Data are presented as means ± SEM (n=4-5 mice per group).

Data information: (B-H) Student's t-test was used for the statistical analysis (*P<0.05).
CoQ Deficiency Causes Disruption of Mitochondrial Sulfide Oxidation, a new Pathomechanism Associated to this Syndrome (2016)
Figure 2

Figure 2. SQR levels and activity depends on CoQ levels in mice tissues.

Sqr mRNA levels (A, B, C), SQR protein levels (D, E, F) and SQR activity (G, H) in cerebrum (A, D), kidneys (B, E, G) and muscle (C, F, H) of Coq9+/+, Coq9R239X and Coq9Q95X mice. Note that SQR western-blots were performed in isolated cerebral mitochondria due to the low levels of this protein in cerebrum. In kidneys and muscle the western-blots were performed in tissue homogenates. Data are expressed as mean ± SD. *P < 0.05; **P < 0.01; ***P < 0.001; Coq9R239X and Coq9Q95X mice versus Coq9+/+ mice. #P < 0.05; ##P < 0.01; ###P < 0.001; Coq9R239X versus Coq9Q95X mice (one-way ANOVA with a Tukey's post hoc test; n = 5-9 for each group).
CoQ Deficiency Causes Disruption of Mitochondrial Sulfide Oxidation, a new Pathomechanism Associated to this Syndrome (2016)
Figure 3

Figure 3. Human skin fibroblasts and mouse tissues with primary CoQ10 deficiency exhibit increased SQR protein levels after exogenous CoQ10 supplementation.

Levels of CoQ10 in fibroblasts of controls (C) and patients (P1-4) with primary CoQ10 deficiency (A). Levels of SQR protein in fibroblasts of controls (C) and patients (P1-4) with primary CoQ10 deficiency cultured without (vehicle) and with 5μM of CoQ10 (+ CoQ10) during 1 day or 7 days (B). Total CoQ levels (CoQ9 + CoQ10) in kidneys (C) and muscle (D) of Coq9+/+, Coq9R239X and Coq9R239X + ubiquinol-10 (Q10H2) mice. Levels of SQR protein in kidneys (E) and muscle (F) of Coq9+/+, Coq9R239X and Coq9R239X + ubiquinol-10 (Q10H2) mice. Data are expressed as mean ± SD. **P < 0.01; ***P < 0.001; patients versus controls, as well as Coq9R239X versus Coq9+/+ mice. +P < 0.05; ++P < 0.01; +++P < 0.001; + CoQ10 versus vehicle (one-way ANOVA with a Tukey's post hoc test; n = 3-5 for each group).
CoQ Deficiency Causes Disruption of Mitochondrial Sulfide Oxidation, a new Pathomechanism Associated to this Syndrome (2016)
Figure 4

Figure 4. Changes in the proteins involved in the mitochondrial sulfide oxidation pathway in response to SQR deficiency in Coq9R239X mice.

TST protein levels in cerebrum (A), kidneys (B) and muscle (C) of Coq9+/+, Coq9R239X and Coq9Q95X mice. TST activity in cerebrum (D), kidneys (E) and muscle (F) of Coq9+/+, Coq9R239X and Coq9Q95X mice. ETEH1 (SDO) protein levels in cerebrum (G), kidneys (H) and muscle (I) of Coq9+/+, Coq9R239X and Coq9Q95X mice. Images of Figures 4C, F and I were obtained from the same membrane after stripping and re-blotting. Data are expressed as mean ± SD. *P < 0.05; **P < 0.01; ***P < 0.001; Coq9R239X and Coq9Q95X mice versus Coq9+/+ mice. ##P < 0.05; ##P < 0.01; ###P < 0.001; Coq9R239X versus Coq9Q95X mice (one-way ANOVA with a Tukey's post hoc test; n = 5-9 for each group).
CoQ Deficiency Causes Disruption of Mitochondrial Sulfide Oxidation, a new Pathomechanism Associated to this Syndrome (2016)
Figure 5

Figure 5. Tissue levels of sulfides in CoQ deficient mice.

Quantification of sulfide levels in cerebrum and kidneys of Coq9+/+, Coq9R239X and Coq9Q95X mice (A). Qualitative measurement of hydrogen sulfide in cerebrum and kidneys of Coq9+/+ and Coq9R239X mice (B). Data are expressed as mean ± SD. *P < 0.05; Coq9R239X and Coq9Q95X mice versus Coq9+/+ mice. ##P < 0.01; Coq9R239X versus Coq9Q95X mice (one-way ANOVA with a Tukey's post hoc test; n = 5-10 for each group).
CoQ Deficiency Causes Disruption of Mitochondrial Sulfide Oxidation, a new Pathomechanism Associated to this Syndrome (2016)
Figure 6

Figure 6. Glutathione system and neurotransmitters biosynthesis are compromised in cerebrum of Coq9R239X mice.

Total GSH in cytosol and mitochondria of cerebrum of Coq9+/+ and Coq9R239X mice (A). Cytosolic GPx and GRd activities in cerebrum of Coq9+/+ and Coq9R239X mice (B). Levels of GPx4 (C) and GRd (D) protein in cerebral homogenate of Coq9+/+ and Coq9R239X mice. Levels of L-Glutamate (L-Glu), N-Acetyl-Glutamate (NacGlu), L-Tryptophan (L-Trp), 5HIAA, N-Acetyl-Tryptophan (NALT), L-Tyrosine (L-Tyr) in cerebrum of Coq9+/+ and Coq9R239X mice (E). Biosynthetic pathway of GSH, serotonin and catecholamine (F). Data are expressed as mean ± SD. *P < 0.05; **P < 0.01; ***P < 0.001; Coq9R239X mice versus Coq9+/+ mice (t test; n = 5 for each group).
CoQ Deficiency Causes Disruption of Mitochondrial Sulfide Oxidation, a new Pathomechanism Associated to this Syndrome (2016)
Figure 7

Figure 7. Supplementation with an H2S donor in wild-type animals induces changes in neurotransmitters levels.

SQR (A) and TST (B) protein levels in human skin fibroblasts supplemented with the H2S donor GYY4137. TST protein level in kidneys (C) and cerebrum (D) of Coq9+/+ mice supplemented with the H2S donor GYY4137. Levels of neurotransmitters in cerebrum of Coq9+/+ mice supplemented with the H2S donor GYY4137 (E). “Images of Figures 7A and B were obtained from the same membrane after stripping and re-blotting”. Data are expressed as mean ± SD. **P < 0.01; ***P < 0.001; Coq9+/+ mice supplemented with GYY4137 versus Coq9+/+ mice. (t test; n = 4-6 for each group).