We use cookies to understand how you use our site and to improve your experience. This includes personalizing content and advertising. To learn more, click here. By continuing to use our site, you accept our use of cookies. Cookie Policy.

Features Partner Sites Information LinkXpress
Sign In
Advertise with Us
LGC Clinical Diagnostics

Download Mobile App




Cell Biologists Find That Certain Mitochondrial Diseases Stem from Coenzyme Q10 Depletion

By LabMedica International staff writers
Posted on 25 Feb 2015
Print article
Image: In mice, mitochondria (green) in healthy (left) and Mfn1-deficient heart muscle cells (center) are organized in a linear arrangement, but the organelles are enlarged and disorganized in Mfn2-deficient cells (right) (Photo courtesy of the Rockefeller Press).
Image: In mice, mitochondria (green) in healthy (left) and Mfn1-deficient heart muscle cells (center) are organized in a linear arrangement, but the organelles are enlarged and disorganized in Mfn2-deficient cells (right) (Photo courtesy of the Rockefeller Press).
A team of German cell biologists has linked the development of certain mitochondrial-linked diseases to depletion of the organelles' pool of coenzyme Q10 brought about by mutation in the MFN2 gene, which encodes the fusion protein mitofusin 2.

Despite the established role of mitofusins (Mfn1 and Mfn2) in mitochondrial fusion, only Mfn2 had been associated with metabolic and neurodegenerative diseases, which suggests that this protein is needed to maintain mitochondrial energy metabolism. Mice lacking the MFN1 gene, which encodes mitofusin 1, seem perfectly healthy, but MFN2-deficient mice die soon after birth. Furthermore, mutations in the MFN2 gene cause human diseases, including the peripheral neuropathy Charcot-Marie-Tooth type 2A. The molecular basis for the mitochondrial dysfunction encountered in the absence of Mfn2 has not been explained.

In the current study, investigators at the Max Planck Institute for Biology of Ageing (Cologne, Germany) worked with cultures of mouse heart muscle cells lacking the MFN2 gene.

They reported in the February 16, 2015, online edition of the Journal of Cell Biology that energy metabolism in the cells was impaired compared to that of healthy heart cells or of heart cells that lacked only Mfn1. The energy metabolic process in the Mfn2-deficient cells was found to have been disrupted by reduced levels of coenzyme Q, a key component of the mitochondrial respiratory chain that generates cellular energy in the form of ATP.

The reduced respiratory chain function in the mitochondria of cells lacking Mfn2 could be partially restored by supplementation with coenzyme Q10, which suggested a possible therapeutic strategy for patients with diseases caused by mutations in the MFN2 gene.

Related Links:

Max Planck Institute for Biology of Ageing


Platinum Member
COVID-19 Rapid Test
OSOM COVID-19 Antigen Rapid Test
HLX
POCT Fluorescent Immunoassay Analyzer
FIA Go
Gold Member
Xylazine Immunoassay Test
Xylazine ELISA

Print article

Channels

Clinical Chemistry

view channel
Image: Reaching speeds up to 6,000 RPM, this centrifuge forms the basis for a new type of inexpensive, POC biomedical test (Photo courtesy of Duke University)

POC Biomedical Test Spins Water Droplet Using Sound Waves for Cancer Detection

Exosomes, tiny cellular bioparticles carrying a specific set of proteins, lipids, and genetic materials, play a crucial role in cell communication and hold promise for non-invasive diagnostics.... Read more

Molecular Diagnostics

view channel
Image: MOF materials efficiently enrich cfDNA and cfRNA in blood through simple operational process (Photo courtesy of Science China Press)

Blood Circulating Nucleic Acid Enrichment Technique Enables Non-Invasive Liver Cancer Diagnosis

The ability to diagnose diseases early can significantly enhance the effectiveness of clinical treatments and improve survival rates. One promising approach for non-invasive early diagnosis is the use... Read more

Hematology

view channel
Image: The low-cost portable device rapidly identifies chemotherapy patients at risk of sepsis (Photo courtesy of 52North Health)

POC Finger-Prick Blood Test Determines Risk of Neutropenic Sepsis in Patients Undergoing Chemotherapy

Neutropenia, a decrease in neutrophils (a type of white blood cell crucial for fighting infections), is a frequent side effect of certain cancer treatments. This condition elevates the risk of infections,... Read more

Pathology

view channel
Image: The OvaCis Rapid Test discriminates benign from malignant epithelial ovarian cysts (Photo courtesy of INEX)

Intra-Operative POC Device Distinguishes Between Benign and Malignant Ovarian Cysts within 15 Minutes

Ovarian cysts represent a significant health issue for women globally, with up to 10% experiencing this condition at some point in their lives. These cysts form when fluid collects within a thin membrane... Read more
Copyright © 2000-2024 Globetech Media. All rights reserved.