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 Experiment Videos

Complex I assembly: a puzzling problem.

Rutger Vogel1, Leo Nijtmans, Cristina Ugalde

  • 1Nijmegen Centre for Mitochondrial Disorders at the Department of Paediatrics, University Medical Centre Nijmegen, Nijmegen, The Netherlands.

Current Opinion in Neurology
|March 17, 2004
PubMed
Summary
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

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

Sort by
Same author

COXFA4L2 upregulation preserves residual cytochrome c oxidase activity in COXFA4-related Leigh-like encephalopathy.

Nature communications·2026
Same author

Impact of compound heterozygous SDHA variants on mitochondrial function in pediatric with neurological disease.

Mitochondrion·2026
Same author

The goal attainment scale in primary mitochondrial disease: Construct validity and lessons learned from a randomized controlled trial.

Molecular genetics and metabolism·2026
Same author

Sensitivity of primary mitochondrial disease fibroblasts to ferroptosis: The role of intracellular iron.

Mitochondrion·2026
Same author

Effect of Pegcetacoplan on Aqueous Humor Proteome in Geographic Atrophy: A Prospective Exploration.

Investigative ophthalmology & visual science·2025
Same author

Retraction notice to "Cytoplasmic gelsolin increases mitochondrial activity and reduces Aβ burden in a mouse model of Alzheimer's disease" [Neurobiology of Disease 36 (2009) 42-50].

Neurobiology of disease·2025
Same journal

Movement disorders and Parkinson's disease: collaborative and interdisciplinary research to advance understanding of neural circuit dysfunction, pathophysiology, and care: new horizons in technology, neuroimaging, neurophysiology, and genetics toward personalized medicine.

Current opinion in neurology·2026
Same journal

Editorial introduction.

Current opinion in neurology·2026
Same journal

Multimodal mapping of balance dysfunction in Parkinson's disease: a consensus roadmap for research and intervention.

Current opinion in neurology·2026
Same journal

Tourette syndrome: brain neurophysiology, circuit dysfunction, and neuromodulation across invasive and noninvasive approaches.

Current opinion in neurology·2026
Same journal

Dystonia: from phenotypes to genetics and therapeutic advances.

Current opinion in neurology·2026
Same journal

What can we learn from eye movements in movement disorders and Parkinson's disease?

Current opinion in neurology·2026
See all related articles

Mitochondrial complex I deficiency is a common cause of fatal disorders. Understanding complex I assembly is crucial for diagnosing and potentially treating these conditions, especially in patients with unknown genetic causes.

Area of Science:

  • Biochemistry
  • Genetics
  • Mitochondrial Biology

Background:

  • Mitochondrial oxidative phosphorylation disorders, particularly complex I deficiency, are often fatal.
  • While mutations in known subunits allow diagnosis, some patients remain undiagnosed.
  • Defects in assembly proteins are suspected in these uncharacterized cases.

Purpose of the Study:

  • To review the current understanding of complex I assembly.
  • To highlight recent developments and future perspectives in the field.
  • To explore the role of accessory subunits in complex I biogenesis.

Main Methods:

  • Review of recent literature on complex I assembly.
  • Analysis of proposed models for human complex I assembly.
  • Investigation of supercomplex assembly and stability.

Related Experiment Videos

Main Results:

  • A model for human complex I assembly has been proposed.
  • Insights into supercomplexes may explain combined deficiencies.
  • Accessory subunits are linked to complex I biogenesis and regulation.

Conclusions:

  • Research on complex I assembly is rapidly advancing.
  • Comparing theoretical and experimental models remains challenging.
  • Understanding assembly will improve diagnosis and potential therapies for mitochondrial disorders.