Review



mitochondrial complex iv activity  (Elabscience Biotechnology)


Bioz Verified Symbol Elabscience Biotechnology is a verified supplier
Bioz Manufacturer Symbol Elabscience Biotechnology manufactures this product  
  • Logo
  • About
  • News
  • Press Release
  • Team
  • Advisors
  • Partners
  • Contact
  • Bioz Stars
  • Bioz vStars
  • 94

    Structured Review

    Elabscience Biotechnology mitochondrial complex iv activity
    LC attenuated myocardial <t>mitochondrial</t> structural damage and respiratory chain abnormalities in EAM mice. (A) Transmission electron microscopy showing morphological changes in mouse heart mitochondria (×5,000, scale bar = 2 μm). Changes in (B) mitochondrial complex IV activity and (C) ATP levels in each group, n = 3. (D,E) DCFH-DA staining flow cytometry to detect ROS levels in each group, n = 3. Data are expressed as Mean ± SEM with individual data points shown. ATP, adenosine triphosphate; ROS, reactive oxygen species.
    Mitochondrial Complex Iv Activity, supplied by Elabscience Biotechnology, used in various techniques. Bioz Stars score: 94/100, based on 7 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
    https://www.bioz.com/result/mitochondrial complex iv activity/product/Elabscience Biotechnology
    Average 94 stars, based on 7 article reviews
    mitochondrial complex iv activity - by Bioz Stars, 2026-06
    94/100 stars

    Images

    1) Product Images from "Levocarnitine improves cardiac energy metabolic remodeling in myocarditis mice"

    Article Title: Levocarnitine improves cardiac energy metabolic remodeling in myocarditis mice

    Journal: Frontiers in Pharmacology

    doi: 10.3389/fphar.2025.1706936

    LC attenuated myocardial mitochondrial structural damage and respiratory chain abnormalities in EAM mice. (A) Transmission electron microscopy showing morphological changes in mouse heart mitochondria (×5,000, scale bar = 2 μm). Changes in (B) mitochondrial complex IV activity and (C) ATP levels in each group, n = 3. (D,E) DCFH-DA staining flow cytometry to detect ROS levels in each group, n = 3. Data are expressed as Mean ± SEM with individual data points shown. ATP, adenosine triphosphate; ROS, reactive oxygen species.
    Figure Legend Snippet: LC attenuated myocardial mitochondrial structural damage and respiratory chain abnormalities in EAM mice. (A) Transmission electron microscopy showing morphological changes in mouse heart mitochondria (×5,000, scale bar = 2 μm). Changes in (B) mitochondrial complex IV activity and (C) ATP levels in each group, n = 3. (D,E) DCFH-DA staining flow cytometry to detect ROS levels in each group, n = 3. Data are expressed as Mean ± SEM with individual data points shown. ATP, adenosine triphosphate; ROS, reactive oxygen species.

    Techniques Used: Transmission Assay, Electron Microscopy, Activity Assay, Staining, Flow Cytometry

    LC improves myocardial mitochondrial substrate utilization in EAM mice. (A) Serum H-FABP levels in each group of mice, n = 3. (B–D) Western blot analysis of OCTN-2 and CPT-1B proteins, n = 3. (E) FFA and (F) LAC levels in myocardial tissue from each group of mice, n = 3. Data are expressed as Mean ± SEM with individual data points shown. FABP, heart-type fatty acid-binding protein; OCTN2, organic carnitine transporter novel type 2; CPT-1B, carnitine palmitoyltransferase-1B; FFA, free fatty acid; LAC, lactic acid.
    Figure Legend Snippet: LC improves myocardial mitochondrial substrate utilization in EAM mice. (A) Serum H-FABP levels in each group of mice, n = 3. (B–D) Western blot analysis of OCTN-2 and CPT-1B proteins, n = 3. (E) FFA and (F) LAC levels in myocardial tissue from each group of mice, n = 3. Data are expressed as Mean ± SEM with individual data points shown. FABP, heart-type fatty acid-binding protein; OCTN2, organic carnitine transporter novel type 2; CPT-1B, carnitine palmitoyltransferase-1B; FFA, free fatty acid; LAC, lactic acid.

    Techniques Used: Western Blot, Binding Assay

    Pearson’s correlation analysis of p-Akt and PGC-1α with mitochondrial metabolic function. Scatter plots showing the correlation between myocardial p-Akt protein expression and (A) COX IV activity, (B) CPT-1B expression, (C) ROS levels, and (D) ATP. Scatter plots showing the correlation between myocardial PGC-1α protein expression and (E) COX IV activity, (F) CPT-1B expression, (G) ROS levels, and (H) ATP. (n = 9 from 3 groups).
    Figure Legend Snippet: Pearson’s correlation analysis of p-Akt and PGC-1α with mitochondrial metabolic function. Scatter plots showing the correlation between myocardial p-Akt protein expression and (A) COX IV activity, (B) CPT-1B expression, (C) ROS levels, and (D) ATP. Scatter plots showing the correlation between myocardial PGC-1α protein expression and (E) COX IV activity, (F) CPT-1B expression, (G) ROS levels, and (H) ATP. (n = 9 from 3 groups).

    Techniques Used: Expressing, Activity Assay



    Similar Products

    94
    Elabscience Biotechnology mitochondrial complex iv activity
    LC attenuated myocardial <t>mitochondrial</t> structural damage and respiratory chain abnormalities in EAM mice. (A) Transmission electron microscopy showing morphological changes in mouse heart mitochondria (×5,000, scale bar = 2 μm). Changes in (B) mitochondrial complex IV activity and (C) ATP levels in each group, n = 3. (D,E) DCFH-DA staining flow cytometry to detect ROS levels in each group, n = 3. Data are expressed as Mean ± SEM with individual data points shown. ATP, adenosine triphosphate; ROS, reactive oxygen species.
    Mitochondrial Complex Iv Activity, supplied by Elabscience Biotechnology, used in various techniques. Bioz Stars score: 94/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
    https://www.bioz.com/result/mitochondrial complex iv activity/product/Elabscience Biotechnology
    Average 94 stars, based on 1 article reviews
    mitochondrial complex iv activity - by Bioz Stars, 2026-06
    94/100 stars
      Buy from Supplier

    94
    Elabscience Biotechnology e bc k837 m
    LC attenuated myocardial <t>mitochondrial</t> structural damage and respiratory chain abnormalities in EAM mice. (A) Transmission electron microscopy showing morphological changes in mouse heart mitochondria (×5,000, scale bar = 2 μm). Changes in (B) mitochondrial complex IV activity and (C) ATP levels in each group, n = 3. (D,E) DCFH-DA staining flow cytometry to detect ROS levels in each group, n = 3. Data are expressed as Mean ± SEM with individual data points shown. ATP, adenosine triphosphate; ROS, reactive oxygen species.
    E Bc K837 M, supplied by Elabscience Biotechnology, used in various techniques. Bioz Stars score: 94/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
    https://www.bioz.com/result/e bc k837 m/product/Elabscience Biotechnology
    Average 94 stars, based on 1 article reviews
    e bc k837 m - by Bioz Stars, 2026-06
    94/100 stars
      Buy from Supplier

    93
    Elabscience Biotechnology micro mitochondrial respiratory chain complex iv activity assay kit
    LC attenuated myocardial <t>mitochondrial</t> structural damage and respiratory chain abnormalities in EAM mice. (A) Transmission electron microscopy showing morphological changes in mouse heart mitochondria (×5,000, scale bar = 2 μm). Changes in (B) mitochondrial complex IV activity and (C) ATP levels in each group, n = 3. (D,E) DCFH-DA staining flow cytometry to detect ROS levels in each group, n = 3. Data are expressed as Mean ± SEM with individual data points shown. ATP, adenosine triphosphate; ROS, reactive oxygen species.
    Micro Mitochondrial Respiratory Chain Complex Iv Activity Assay Kit, supplied by Elabscience Biotechnology, used in various techniques. Bioz Stars score: 93/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
    https://www.bioz.com/result/micro mitochondrial respiratory chain complex iv activity assay kit/product/Elabscience Biotechnology
    Average 93 stars, based on 1 article reviews
    micro mitochondrial respiratory chain complex iv activity assay kit - by Bioz Stars, 2026-06
    93/100 stars
      Buy from Supplier

    94
    Elabscience Biotechnology cytochrome c oxidase activity assay kit
    LC attenuated myocardial <t>mitochondrial</t> structural damage and respiratory chain abnormalities in EAM mice. (A) Transmission electron microscopy showing morphological changes in mouse heart mitochondria (×5,000, scale bar = 2 μm). Changes in (B) mitochondrial complex IV activity and (C) ATP levels in each group, n = 3. (D,E) DCFH-DA staining flow cytometry to detect ROS levels in each group, n = 3. Data are expressed as Mean ± SEM with individual data points shown. ATP, adenosine triphosphate; ROS, reactive oxygen species.
    Cytochrome C Oxidase Activity Assay Kit, supplied by Elabscience Biotechnology, used in various techniques. Bioz Stars score: 94/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
    https://www.bioz.com/result/cytochrome c oxidase activity assay kit/product/Elabscience Biotechnology
    Average 94 stars, based on 1 article reviews
    cytochrome c oxidase activity assay kit - by Bioz Stars, 2026-06
    94/100 stars
      Buy from Supplier

    94
    Elabscience Biotechnology complex iv
    LC attenuated myocardial <t>mitochondrial</t> structural damage and respiratory chain abnormalities in EAM mice. (A) Transmission electron microscopy showing morphological changes in mouse heart mitochondria (×5,000, scale bar = 2 μm). Changes in (B) mitochondrial complex IV activity and (C) ATP levels in each group, n = 3. (D,E) DCFH-DA staining flow cytometry to detect ROS levels in each group, n = 3. Data are expressed as Mean ± SEM with individual data points shown. ATP, adenosine triphosphate; ROS, reactive oxygen species.
    Complex Iv, supplied by Elabscience Biotechnology, used in various techniques. Bioz Stars score: 94/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
    https://www.bioz.com/result/complex iv/product/Elabscience Biotechnology
    Average 94 stars, based on 1 article reviews
    complex iv - by Bioz Stars, 2026-06
    94/100 stars
      Buy from Supplier

    94
    Elabscience Biotechnology cell mitochondrial complex iv
    Fig. 3 Dysfunction of mitochondria in iPSC-derived neurons due to mutation in TIMM8A. A, B No differences in HSP60, TOMM40, and TIMM23 levels were observed among CTRL-, MUT-, and MTS-neurons by Western blot. Relative protein levels were quantified and normalized to β-Tubulin as a loading control. n = 3 independent biological replicates. C, D. Western blot analysis showed decreased levels of COX4 in <t>mitochondrial</t> complex IV in MUT- and MTS-neurons. Relative levels of COX4 were quantified and normalized to HSP60 as a loading control. n = 3 independent biological replicates. E The activity of mitochondrial complex IV was decreased in MUT- and MTS-neurons compared to CTRL-neurons. n = 3 independent biological replicates. F ATP production was decreased in MUT- and MTS-neurons compared to CTRL- neurons. n = 3 independent biological replicates. G ROS levels were increased in MUT- and MTS-neurons compared to CTRL-neurons. n = 3 independent biological replicates. H, I Levels of Cyt c and cleaved caspase-3 in CTRL-, MUT-, and MTS-neurons after treatment with 100 µM TBHP for 24 h were analyzed by Western blot. Relative protein levels of Cyt c and cleaved caspase-3 in the cytosol were quantified and normalized to β-Tubulin as a loading control. n = 3 independent biological replicates. Data are represented as mean ± SD. Significance was determined by one-way ANOVA with Tukey’s post hoc test: ns, not significant; *p < 0.05; **p < 0.01; ****p < 0.0001 (applied to panels B, D–G, and I).
    Cell Mitochondrial Complex Iv, supplied by Elabscience Biotechnology, used in various techniques. Bioz Stars score: 94/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
    https://www.bioz.com/result/cell mitochondrial complex iv/product/Elabscience Biotechnology
    Average 94 stars, based on 1 article reviews
    cell mitochondrial complex iv - by Bioz Stars, 2026-06
    94/100 stars
      Buy from Supplier

    94
    Elabscience Biotechnology cytochrome c oxidase activity
    Fig. 3 Dysfunction of mitochondria in iPSC-derived neurons due to mutation in TIMM8A. A, B No differences in HSP60, TOMM40, and TIMM23 levels were observed among CTRL-, MUT-, and MTS-neurons by Western blot. Relative protein levels were quantified and normalized to β-Tubulin as a loading control. n = 3 independent biological replicates. C, D. Western blot analysis showed decreased levels of COX4 in <t>mitochondrial</t> complex IV in MUT- and MTS-neurons. Relative levels of COX4 were quantified and normalized to HSP60 as a loading control. n = 3 independent biological replicates. E The activity of mitochondrial complex IV was decreased in MUT- and MTS-neurons compared to CTRL-neurons. n = 3 independent biological replicates. F ATP production was decreased in MUT- and MTS-neurons compared to CTRL- neurons. n = 3 independent biological replicates. G ROS levels were increased in MUT- and MTS-neurons compared to CTRL-neurons. n = 3 independent biological replicates. H, I Levels of Cyt c and cleaved caspase-3 in CTRL-, MUT-, and MTS-neurons after treatment with 100 µM TBHP for 24 h were analyzed by Western blot. Relative protein levels of Cyt c and cleaved caspase-3 in the cytosol were quantified and normalized to β-Tubulin as a loading control. n = 3 independent biological replicates. Data are represented as mean ± SD. Significance was determined by one-way ANOVA with Tukey’s post hoc test: ns, not significant; *p < 0.05; **p < 0.01; ****p < 0.0001 (applied to panels B, D–G, and I).
    Cytochrome C Oxidase Activity, supplied by Elabscience Biotechnology, used in various techniques. Bioz Stars score: 94/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
    https://www.bioz.com/result/cytochrome c oxidase activity/product/Elabscience Biotechnology
    Average 94 stars, based on 1 article reviews
    cytochrome c oxidase activity - by Bioz Stars, 2026-06
    94/100 stars
      Buy from Supplier

    Image Search Results


    LC attenuated myocardial mitochondrial structural damage and respiratory chain abnormalities in EAM mice. (A) Transmission electron microscopy showing morphological changes in mouse heart mitochondria (×5,000, scale bar = 2 μm). Changes in (B) mitochondrial complex IV activity and (C) ATP levels in each group, n = 3. (D,E) DCFH-DA staining flow cytometry to detect ROS levels in each group, n = 3. Data are expressed as Mean ± SEM with individual data points shown. ATP, adenosine triphosphate; ROS, reactive oxygen species.

    Journal: Frontiers in Pharmacology

    Article Title: Levocarnitine improves cardiac energy metabolic remodeling in myocarditis mice

    doi: 10.3389/fphar.2025.1706936

    Figure Lengend Snippet: LC attenuated myocardial mitochondrial structural damage and respiratory chain abnormalities in EAM mice. (A) Transmission electron microscopy showing morphological changes in mouse heart mitochondria (×5,000, scale bar = 2 μm). Changes in (B) mitochondrial complex IV activity and (C) ATP levels in each group, n = 3. (D,E) DCFH-DA staining flow cytometry to detect ROS levels in each group, n = 3. Data are expressed as Mean ± SEM with individual data points shown. ATP, adenosine triphosphate; ROS, reactive oxygen species.

    Article Snippet: Mitochondrial complex IV activity was measured with the kit (E-BC-K837-M, Elabscience, Wuhan, China) to assess the integrity of the mitochondrial respiratory chain.

    Techniques: Transmission Assay, Electron Microscopy, Activity Assay, Staining, Flow Cytometry

    LC improves myocardial mitochondrial substrate utilization in EAM mice. (A) Serum H-FABP levels in each group of mice, n = 3. (B–D) Western blot analysis of OCTN-2 and CPT-1B proteins, n = 3. (E) FFA and (F) LAC levels in myocardial tissue from each group of mice, n = 3. Data are expressed as Mean ± SEM with individual data points shown. FABP, heart-type fatty acid-binding protein; OCTN2, organic carnitine transporter novel type 2; CPT-1B, carnitine palmitoyltransferase-1B; FFA, free fatty acid; LAC, lactic acid.

    Journal: Frontiers in Pharmacology

    Article Title: Levocarnitine improves cardiac energy metabolic remodeling in myocarditis mice

    doi: 10.3389/fphar.2025.1706936

    Figure Lengend Snippet: LC improves myocardial mitochondrial substrate utilization in EAM mice. (A) Serum H-FABP levels in each group of mice, n = 3. (B–D) Western blot analysis of OCTN-2 and CPT-1B proteins, n = 3. (E) FFA and (F) LAC levels in myocardial tissue from each group of mice, n = 3. Data are expressed as Mean ± SEM with individual data points shown. FABP, heart-type fatty acid-binding protein; OCTN2, organic carnitine transporter novel type 2; CPT-1B, carnitine palmitoyltransferase-1B; FFA, free fatty acid; LAC, lactic acid.

    Article Snippet: Mitochondrial complex IV activity was measured with the kit (E-BC-K837-M, Elabscience, Wuhan, China) to assess the integrity of the mitochondrial respiratory chain.

    Techniques: Western Blot, Binding Assay

    Pearson’s correlation analysis of p-Akt and PGC-1α with mitochondrial metabolic function. Scatter plots showing the correlation between myocardial p-Akt protein expression and (A) COX IV activity, (B) CPT-1B expression, (C) ROS levels, and (D) ATP. Scatter plots showing the correlation between myocardial PGC-1α protein expression and (E) COX IV activity, (F) CPT-1B expression, (G) ROS levels, and (H) ATP. (n = 9 from 3 groups).

    Journal: Frontiers in Pharmacology

    Article Title: Levocarnitine improves cardiac energy metabolic remodeling in myocarditis mice

    doi: 10.3389/fphar.2025.1706936

    Figure Lengend Snippet: Pearson’s correlation analysis of p-Akt and PGC-1α with mitochondrial metabolic function. Scatter plots showing the correlation between myocardial p-Akt protein expression and (A) COX IV activity, (B) CPT-1B expression, (C) ROS levels, and (D) ATP. Scatter plots showing the correlation between myocardial PGC-1α protein expression and (E) COX IV activity, (F) CPT-1B expression, (G) ROS levels, and (H) ATP. (n = 9 from 3 groups).

    Article Snippet: Mitochondrial complex IV activity was measured with the kit (E-BC-K837-M, Elabscience, Wuhan, China) to assess the integrity of the mitochondrial respiratory chain.

    Techniques: Expressing, Activity Assay

    Fig. 3 Dysfunction of mitochondria in iPSC-derived neurons due to mutation in TIMM8A. A, B No differences in HSP60, TOMM40, and TIMM23 levels were observed among CTRL-, MUT-, and MTS-neurons by Western blot. Relative protein levels were quantified and normalized to β-Tubulin as a loading control. n = 3 independent biological replicates. C, D. Western blot analysis showed decreased levels of COX4 in mitochondrial complex IV in MUT- and MTS-neurons. Relative levels of COX4 were quantified and normalized to HSP60 as a loading control. n = 3 independent biological replicates. E The activity of mitochondrial complex IV was decreased in MUT- and MTS-neurons compared to CTRL-neurons. n = 3 independent biological replicates. F ATP production was decreased in MUT- and MTS-neurons compared to CTRL- neurons. n = 3 independent biological replicates. G ROS levels were increased in MUT- and MTS-neurons compared to CTRL-neurons. n = 3 independent biological replicates. H, I Levels of Cyt c and cleaved caspase-3 in CTRL-, MUT-, and MTS-neurons after treatment with 100 µM TBHP for 24 h were analyzed by Western blot. Relative protein levels of Cyt c and cleaved caspase-3 in the cytosol were quantified and normalized to β-Tubulin as a loading control. n = 3 independent biological replicates. Data are represented as mean ± SD. Significance was determined by one-way ANOVA with Tukey’s post hoc test: ns, not significant; *p < 0.05; **p < 0.01; ****p < 0.0001 (applied to panels B, D–G, and I).

    Journal: Cell death & disease

    Article Title: CHCHD2 rescues the mitochondrial dysfunction in iPSC-derived neurons from patient with Mohr-Tranebjaerg syndrome.

    doi: 10.1038/s41419-025-07472-9

    Figure Lengend Snippet: Fig. 3 Dysfunction of mitochondria in iPSC-derived neurons due to mutation in TIMM8A. A, B No differences in HSP60, TOMM40, and TIMM23 levels were observed among CTRL-, MUT-, and MTS-neurons by Western blot. Relative protein levels were quantified and normalized to β-Tubulin as a loading control. n = 3 independent biological replicates. C, D. Western blot analysis showed decreased levels of COX4 in mitochondrial complex IV in MUT- and MTS-neurons. Relative levels of COX4 were quantified and normalized to HSP60 as a loading control. n = 3 independent biological replicates. E The activity of mitochondrial complex IV was decreased in MUT- and MTS-neurons compared to CTRL-neurons. n = 3 independent biological replicates. F ATP production was decreased in MUT- and MTS-neurons compared to CTRL- neurons. n = 3 independent biological replicates. G ROS levels were increased in MUT- and MTS-neurons compared to CTRL-neurons. n = 3 independent biological replicates. H, I Levels of Cyt c and cleaved caspase-3 in CTRL-, MUT-, and MTS-neurons after treatment with 100 µM TBHP for 24 h were analyzed by Western blot. Relative protein levels of Cyt c and cleaved caspase-3 in the cytosol were quantified and normalized to β-Tubulin as a loading control. n = 3 independent biological replicates. Data are represented as mean ± SD. Significance was determined by one-way ANOVA with Tukey’s post hoc test: ns, not significant; *p < 0.05; **p < 0.01; ****p < 0.0001 (applied to panels B, D–G, and I).

    Article Snippet: Activity analysis of mitochondrial complex IV The activity of mitochondrial complex IV was measured using the Cell Mitochondrial Complex IV (Cytochrome C Oxidase) Activity Assay Kit (Elabscience, Cat# E-BC-K837-M) according to the manufacturer’s protocol.

    Techniques: Derivative Assay, Mutagenesis, Western Blot, Control, Activity Assay

    Fig. 4 Altered gene expression in iPSC-derived neurons revealed by RNA sequencing. A A total of 2,843 genes were significantly upregulated, and 1,667 genes were significantly downregulated in MTS-neurons compared to CTRL-neurons (adjusted p < 0.05 and |log2(fold change)| > 1). Top altered genes, including CHCHD2, are labeled. B Top 10 altered biological processes in MTS-neurons compared to CTRL-neurons, identified by Gene Ontology (GO) enrichment analysis (adjusted p < 0.05). C Top 10 altered Kyoto Encyclopedia of Genes and Genomes (KEGG) pathways in MTS-neurons compared to CTRL-neurons (adjusted p < 0.05). D Representative differentially expressed genes involved in mitochondria-related apoptosis. E Western blot analysis of the anti-apoptotic protein BCL-2 and the pro-apoptotic protein BAX in isolated mitochondrial lysates from CTRL- and MTS-neurons. F Western blot analysis of CHCHD2 in isolated mitochondrial lysates from CTRL-, MUT-, and MTS-neurons. G Expression levels of BCL-2 and BAX were quantified and normalized to HSP60 as a loading control. n = 3 independent biological replicates. Data are represented as mean ± SD. Significance was determined by two-tailed unpaired Student’s t test: ns, not significant; **p < 0.01. H CHCHD2 expression levels were quantified and normalized to HSP60 as a loading control. n = 3 independent biological replicates. Data are represented as mean ± SD. Significance was determined by one-way ANOVA with Tukey’s post hoc test: **p < 0.01; ****p < 0.0001.

    Journal: Cell death & disease

    Article Title: CHCHD2 rescues the mitochondrial dysfunction in iPSC-derived neurons from patient with Mohr-Tranebjaerg syndrome.

    doi: 10.1038/s41419-025-07472-9

    Figure Lengend Snippet: Fig. 4 Altered gene expression in iPSC-derived neurons revealed by RNA sequencing. A A total of 2,843 genes were significantly upregulated, and 1,667 genes were significantly downregulated in MTS-neurons compared to CTRL-neurons (adjusted p < 0.05 and |log2(fold change)| > 1). Top altered genes, including CHCHD2, are labeled. B Top 10 altered biological processes in MTS-neurons compared to CTRL-neurons, identified by Gene Ontology (GO) enrichment analysis (adjusted p < 0.05). C Top 10 altered Kyoto Encyclopedia of Genes and Genomes (KEGG) pathways in MTS-neurons compared to CTRL-neurons (adjusted p < 0.05). D Representative differentially expressed genes involved in mitochondria-related apoptosis. E Western blot analysis of the anti-apoptotic protein BCL-2 and the pro-apoptotic protein BAX in isolated mitochondrial lysates from CTRL- and MTS-neurons. F Western blot analysis of CHCHD2 in isolated mitochondrial lysates from CTRL-, MUT-, and MTS-neurons. G Expression levels of BCL-2 and BAX were quantified and normalized to HSP60 as a loading control. n = 3 independent biological replicates. Data are represented as mean ± SD. Significance was determined by two-tailed unpaired Student’s t test: ns, not significant; **p < 0.01. H CHCHD2 expression levels were quantified and normalized to HSP60 as a loading control. n = 3 independent biological replicates. Data are represented as mean ± SD. Significance was determined by one-way ANOVA with Tukey’s post hoc test: **p < 0.01; ****p < 0.0001.

    Article Snippet: Activity analysis of mitochondrial complex IV The activity of mitochondrial complex IV was measured using the Cell Mitochondrial Complex IV (Cytochrome C Oxidase) Activity Assay Kit (Elabscience, Cat# E-BC-K837-M) according to the manufacturer’s protocol.

    Techniques: Gene Expression, Derivative Assay, RNA Sequencing, Labeling, Western Blot, Isolation, Expressing, Control, Two Tailed Test

    Fig. 6 CHCHD2 overexpression rescues mitochondrial morphology defects in iPSC-derived neurons. A, B Mitochondrial morphology in CTRL- and MTS-neurons at day 30, as revealed by transmission electron microscopy (TEM). Scale bar: 1 μm. n = 3 independent biological replicates. Quantification of mitochondrial perimeter, area, aspect ratio, and form factor was performed (103 mitochondria for CTRL group; 102 for MTS group). C, D Mitochondrial morphology in the Vector group and CHCHD2-Flag group of MTS-neurons at day 30, as revealed by TEM. Scale bar: 1 μm. n = 3 independent biological replicates. Quantification of mitochondrial perimeter, area, aspect ratio, and form factor was performed (107 mitochondria for Vector group; 124 for CHCHD2-Flag group). Data are represented as mean ± SD. Significance was determined by two-tailed unpaired Student’s t test: **p < 0.01; ****p < 0.0001.

    Journal: Cell death & disease

    Article Title: CHCHD2 rescues the mitochondrial dysfunction in iPSC-derived neurons from patient with Mohr-Tranebjaerg syndrome.

    doi: 10.1038/s41419-025-07472-9

    Figure Lengend Snippet: Fig. 6 CHCHD2 overexpression rescues mitochondrial morphology defects in iPSC-derived neurons. A, B Mitochondrial morphology in CTRL- and MTS-neurons at day 30, as revealed by transmission electron microscopy (TEM). Scale bar: 1 μm. n = 3 independent biological replicates. Quantification of mitochondrial perimeter, area, aspect ratio, and form factor was performed (103 mitochondria for CTRL group; 102 for MTS group). C, D Mitochondrial morphology in the Vector group and CHCHD2-Flag group of MTS-neurons at day 30, as revealed by TEM. Scale bar: 1 μm. n = 3 independent biological replicates. Quantification of mitochondrial perimeter, area, aspect ratio, and form factor was performed (107 mitochondria for Vector group; 124 for CHCHD2-Flag group). Data are represented as mean ± SD. Significance was determined by two-tailed unpaired Student’s t test: **p < 0.01; ****p < 0.0001.

    Article Snippet: Activity analysis of mitochondrial complex IV The activity of mitochondrial complex IV was measured using the Cell Mitochondrial Complex IV (Cytochrome C Oxidase) Activity Assay Kit (Elabscience, Cat# E-BC-K837-M) according to the manufacturer’s protocol.

    Techniques: Over Expression, Derivative Assay, Transmission Assay, Electron Microscopy, Plasmid Preparation, Two Tailed Test