Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

Animal Mitochondrial Genetics02:59

Animal Mitochondrial Genetics

9.2K
Among all the organelles in an animal cell, only mitochondria have their own independent genomes. Animal mitochondrial DNA is a double-stranded, closed-circular molecule with around 20,000 base pairs. Mitochondrial DNA is unique in that one of its two strands, the heavy, or H, -strand is guanine rich, whereas the complementary strand is cytosine rich and called the light, or L, -strand. Compared to nuclear DNA, mitochondrial DNA has a very low percentage of non-coding regions and is marked by...
9.2K
Comparing Mitochondrial, Chloroplast, and Prokaryotic Genomes02:16

Comparing Mitochondrial, Chloroplast, and Prokaryotic Genomes

16.0K
The present-day mitochondrial and chloroplast genomes have retained some of the characteristics of their ancestral prokaryotes and also have acquired new attributes during their evolution within eukaryotic cells. Like prokaryotic genomes, mitochondrial and chloroplast genomes neither bind with histone-like proteins nor show complex packaging into chromosome-like structures, as observed in eukaryotes. Unlike mitotic cell divisions observed in eukaryotic cells, mitochondria and chloroplasts...
16.0K
Export of Mitochondrial and Chloroplast Genes02:19

Export of Mitochondrial and Chloroplast Genes

4.2K
A eukaryotic cell can have up to three different types of genetic systems: nuclear, mitochondrial, and chloroplast. During evolution, organelles have exported many genes to the nucleus; this transfer is still ongoing in some plant species. Approximately 18% of the Arabidopsis thaliana nuclear genome is thought to be derived from the chloroplast’s cyanobacterial ancestor, and around 75% of the yeast genome derived from the mitochondria’s bacterial ancestor. This export has occurred...
4.2K
The Inner Mitochondrial Membrane01:28

The Inner Mitochondrial Membrane

4.7K
The inner mitochondrial membrane is the primary site of ATP synthesis. The inner membrane domain that forms a smooth layer adjacent to the outer membrane is called the inner boundary membrane. This domain contains membrane transporters that drive metabolites in and out of the mitochondria.  In contrast, the inner membrane network that invaginates into the matrix space is called the cristae membrane. This domain accounts for principle mitochondrial function as it accommodates the protein...
4.7K
Nephrotic Syndrome I : Introduction01:24

Nephrotic Syndrome I : Introduction

623
Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of...
623
Acute Coronary Syndrome I: Introduction01:30

Acute Coronary Syndrome I: Introduction

958
Acute Coronary Syndrome (ACS) encompasses a spectrum of heart conditions caused by sudden obstruction of coronary arteries, typically resulting from the rupture of an atherosclerotic plaque and subsequent thrombus (blood clot) formation. This obstruction can lead to partial or complete blockage of blood flow, causing varying degrees of myocardial ischemia or infarction.ACS includes the following clinical entities:Unstable Angina (UA)Non-ST-Elevation Myocardial Infarction (NSTEMI)ST-Elevation...
958

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

SLC25A3 exports mitochondrial copper to metalate cytochrome <i>c</i> oxidase and prevent cuproptosis.

Proceedings of the National Academy of Sciences of the United States of America·2026
Same author

A broad-spectrum inhibitor of copper-exporting P<sub>1B</sub>-type ATPases.

Proceedings of the National Academy of Sciences of the United States of America·2026
Same author

Noncanonical role of MTP-18 in mitochondrial function and aging via electron transport chain interactions in Caenorhabditis elegans.

Biogerontology·2026
Same author

Cognate amino acid therapies provide preclinical benefit in 19 <i>C. elegans</i> models of ARS2 deficiency.

bioRxiv : the preprint server for biology·2026
Same author

Nanomaterial-induced mitochondrial biogenesis enhances intercellular mitochondrial transfer efficiency.

Proceedings of the National Academy of Sciences of the United States of America·2025
Same author

ndufs2<sup>-/-</sup> zebrafish have impaired survival, neuromuscular activity, morphology, and one-carbon metabolism treatable with folic acid.

bioRxiv : the preprint server for biology·2025

Related Experiment Video

Updated: Jan 29, 2026

Fingerprinting Cardiolipin in Leukocytes by Mass Spectrometry for a Rapid Diagnosis of Barth Syndrome
06:48

Fingerprinting Cardiolipin in Leukocytes by Mass Spectrometry for a Rapid Diagnosis of Barth Syndrome

Published on: March 23, 2022

2.7K

Mitochondrial dysfunctions in barth syndrome.

Sagnika Ghosh1, Donna M Iadarola1, Writoban Basu Ball1

  • 1Department of Biochemistry and Biophysics, Texas A&M University, College Station, TX, USA.

IUBMB Life
|February 13, 2019
PubMed
Summary

Barth syndrome (BTHS), caused by TAZ gene mutations, disrupts mitochondrial cardiolipin remodeling. This review explores how cardiolipin dysfunction impacts mitochondrial health and BTHS pathology across various models.

Keywords:
Barth syndromecardiolipinmitochondria

More Related Videos

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model
06:05

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model

Published on: March 9, 2022

4.4K
Genotyping Single Nucleotide Polymorphisms in the Mitochondrial Genome by Pyrosequencing
07:24

Genotyping Single Nucleotide Polymorphisms in the Mitochondrial Genome by Pyrosequencing

Published on: February 10, 2023

2.0K

Related Experiment Videos

Last Updated: Jan 29, 2026

Fingerprinting Cardiolipin in Leukocytes by Mass Spectrometry for a Rapid Diagnosis of Barth Syndrome
06:48

Fingerprinting Cardiolipin in Leukocytes by Mass Spectrometry for a Rapid Diagnosis of Barth Syndrome

Published on: March 23, 2022

2.7K
An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model
06:05

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model

Published on: March 9, 2022

4.4K
Genotyping Single Nucleotide Polymorphisms in the Mitochondrial Genome by Pyrosequencing
07:24

Genotyping Single Nucleotide Polymorphisms in the Mitochondrial Genome by Pyrosequencing

Published on: February 10, 2023

2.0K

Area of Science:

  • Biochemistry
  • Genetics
  • Cell Biology

Background:

  • Barth syndrome (BTHS) is a rare genetic disorder stemming from mutations in the TAZ gene.
  • TAZ encodes a mitochondrial enzyme crucial for cardiolipin remodeling, a phospholipid vital for mitochondrial membranes.
  • BTHS clinical manifestations arise from impaired mitochondrial functions dependent on remodeled cardiolipin.

Purpose of the Study:

  • To review recent findings on Barth syndrome (BTHS) using diverse genetic models.
  • To elucidate the specific roles of cardiolipin in various mitochondrial functions.
  • To connect cardiolipin-dependent mitochondrial dysfunction to BTHS pathology.

Main Methods:

  • Analysis of findings from yeast models of cardiolipin deficiency.
  • Examination of data from higher eukaryotic models of BTHS.
  • Review of literature on TAZ gene mutations and their functional consequences.

Main Results:

  • Yeast models revealed cardiolipin's essential roles in mitochondrial respiratory chain biogenesis, bioenergetics, metabolism, dynamics, and quality control.
  • Higher eukaryotic models demonstrate a link between mitochondrial dysfunction and tissue/organ impairment in BTHS.
  • The study highlights the multifaceted impact of cardiolipin remodeling defects in BTHS.

Conclusions:

  • Cardiolipin remodeling is critical for multiple mitochondrial functions.
  • Dysfunctional cardiolipin metabolism significantly contributes to Barth syndrome pathology.
  • Understanding these mechanisms across different models is key to addressing BTHS.