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

Mitochondrial Membranes01:45

Mitochondrial Membranes

11.8K
A single mitochondrion is a bean-shaped organelle enclosed by a double-membrane system. The outer membrane of mitochondria is smooth and contains many porins - the integral membrane transporters. Porins enable free diffusion of ions and small uncharged molecules through the outer mitochondrial membrane but limit the transport of molecules larger than 5000 Daltons. Further, the outer mitochondrial membrane forms a unique structure called membrane contact sites with other subcellular organelles,...
11.8K
The Inner Mitochondrial Membrane01:28

The Inner Mitochondrial Membrane

3.5K
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...
3.5K
Translocation of Proteins into the Mitochondria01:19

Translocation of Proteins into the Mitochondria

3.2K
Mitochondrial precursors are translocated to the internal subcompartments via independent mechanisms involving distinct protein machineries called translocases.
Sorting of outer membrane proteins:
Mitochondrial outer membrane proteins are of two types: the transmembrane, beta-barrel porins, and the membrane-anchored, alpha-helical proteins. Beta-barrel porin precursors are translocated by the TOM complex and inserted into the outer mitochondrial membrane by the SAM complex. In contrast,...
3.2K
Mitochondria01:37

Mitochondria

14.2K
Mitochondria are eukaryotic cellular organelles that are known to produce energy through a process called oxidative phosphorylation. Besides their primary function, mitochondria are involved in various cellular processes, including cell growth, differentiation, signaling, metabolism, and senescence. Age-related changes cause a decline in mitochondrial quality and integrity due to increased mitochondrial mutations and oxidative damage. Thus, aging can severely impact mitochondrial functions,...
14.2K
Porin Insertion in the Outer Mitochondrial Membrane01:12

Porin Insertion in the Outer Mitochondrial Membrane

3.2K
Porins are beta-barrel proteins translocated to the mitochondrial outer membrane through the TOM complex into the intermembrane space. Porin precursors bind TIM chaperones within the intermembrane space and are guided to the Sorting and Assembly Machinery complex or SAM complex on the outer mitochondrial membrane.
Three models describe the assembly of porins by the SAM complex and their insertion into the outer membrane. Model 1 suggests that porins are assembled outside the SAM channel as the...
3.2K
Mitochondrial Protein Sorting01:39

Mitochondrial Protein Sorting

4.4K
Mitochondria are double-membrane organelles of the eukaryotes involved in cellular metabolism, signaling, ATP synthesis, and programmed cell death.  Each of these processes requires specific proteins and enzymes that must be correctly sorted to the right mitochondrial subcompartment for the proper functioning of the organelle.
Most of these mitochondrial proteins are encoded by the nucleus and imported to the mitochondria as unfolded or loosely folded precursors. Mitochondrial precursors...
4.4K

You might also read

Related Articles

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

Sort by
Same author

Increased Brown Adipose Tissue Thermogenesis in Phenylketonuria.

MedComm·2026
Same author

Metabolic Reprogramming Coordinates Mannose and Glutamine Metabolism to Maintain Glucose Homeostasis During Glycosuria.

bioRxiv : the preprint server for biology·2026
Same author

Pharmacological administration of FGF21 reverses obesity through a parabrachial-projecting neuron population in the hindbrain.

Cell reports·2026
Same author

Harnessing AlphaFold3 to elucidate BBSome structure and protein partners.

American journal of physiology. Cell physiology·2026
Same author

Genetic deletion of ASIC3 alters left ventricular remodeling and autonomic function after myocardial infarction in mice.

Physiological reports·2026
Same author

A Lamp2a-linked RNA secreted by ADSCs prevents ENO1-lactylation-glycolysis feedback and cell malignant behavior in triple-negative breast cancer.

Cell death & disease·2026
Same journal

Chronic semaglutide alters ingestive behavior without impairing taste function in mice.

Molecular metabolism·2026
Same journal

RNASET2 degrades mRNAs that protect against lipotoxicity.

Molecular metabolism·2026
Same journal

Corrigendum to "Beta-hydroxybutyrate counteracts the deleterious effects of a saturated high-fat diet on synaptic AMPAR receptors and cognitive performance" [Mol Metabol (2025) 102207].

Molecular metabolism·2026
Same journal

Heterogeneous expression patterns of the T2D-associated kinesin-4 KIF21A in pancreatic islet endocrine cells.

Molecular metabolism·2026
Same journal

Skeletal muscle-specific deficiency of Rab geranylgeranyl transferase beta subunit induces myopathy and exacerbates the symptoms caused by HMG-CoA reductase deficiency in mice.

Molecular metabolism·2026
Same journal

Prenatal metabolic adversity reprograms insulin-responsive transcription in the developing nucleus accumbens.

Molecular metabolism·2026
See all related articles

Related Experiment Video

Updated: Aug 17, 2025

Studying Mitochondrial Structure and Function in Drosophila Ovaries
09:53

Studying Mitochondrial Structure and Function in Drosophila Ovaries

Published on: January 4, 2017

24.2K

The BBSome regulates mitochondria dynamics and function.

Deng-Fu Guo1, Ronald A Merrill2, Lan Qian3

  • 1Department of Neuroscience and Pharmacology, University of Iowa Carver College of Medicine, Iowa City, IA, USA; Veterans Affairs Health Care System, Iowa City, IA, USA.

Molecular Metabolism
|December 13, 2022
PubMed
Summary
This summary is machine-generated.

The Bardet-Biedl syndrome (BBS) complex regulates mitochondria function and dynamics. Loss of BBSome causes mitochondrial defects, leading to BBS symptoms, which can be improved by modulating DRP1 activity.

Keywords:
Bardet-biedl syndrome proteinsBody weightLeptin sensitivityMitochondria

More Related Videos

A Model Membrane Platform for Reconstituting Mitochondrial Membrane Dynamics
10:31

A Model Membrane Platform for Reconstituting Mitochondrial Membrane Dynamics

Published on: September 2, 2020

7.7K
Visualizing Mitophagy with Fluorescent Dyes for Mitochondria and Lysosome
07:56

Visualizing Mitophagy with Fluorescent Dyes for Mitochondria and Lysosome

Published on: November 30, 2022

4.7K

Related Experiment Videos

Last Updated: Aug 17, 2025

Studying Mitochondrial Structure and Function in Drosophila Ovaries
09:53

Studying Mitochondrial Structure and Function in Drosophila Ovaries

Published on: January 4, 2017

24.2K
A Model Membrane Platform for Reconstituting Mitochondrial Membrane Dynamics
10:31

A Model Membrane Platform for Reconstituting Mitochondrial Membrane Dynamics

Published on: September 2, 2020

7.7K
Visualizing Mitophagy with Fluorescent Dyes for Mitochondria and Lysosome
07:56

Visualizing Mitophagy with Fluorescent Dyes for Mitochondria and Lysosome

Published on: November 30, 2022

4.7K

Area of Science:

  • Mitochondrial biology
  • Cellular and molecular biology
  • Genetics and disease mechanisms

Background:

  • Mitochondria are crucial for cellular metabolism and physiological functions.
  • The Bardet-Biedl syndrome (BBS) complex, comprising eight BBS proteins, is implicated in various cellular processes.
  • Understanding BBSome's role in mitochondrial regulation is essential for BBS pathophysiology.

Purpose of the Study:

  • To investigate the role of the BBSome in controlling mitochondrial dynamics and function.
  • To elucidate the mechanisms by which BBSome influences mitochondrial health.
  • To explore therapeutic targets for BBS-related mitochondrial dysfunction.

Main Methods:

  • Generated a Bbs1 gene knockout hypothalamic N39 neuronal cell line using CRISPR/Cas9.
  • Analyzed BBSome-deficient mice, with and without AKAP1 gene deletion.
  • Utilized a multidisciplinary approach including cell lines, patient-derived fibroblasts, and mouse models.

Main Results:

  • BBSome disruption leads to mitochondrial hyperfusion and functional abnormalities, including reduced oxygen consumption and altered calcium handling.
  • BBSome regulates dynamin-like protein 1 (DRP1) activity by controlling its phosphorylation and mitochondrial translocation.
  • Deletion of AKAP1 normalized mitochondrial defects and improved BBS-related phenotypes like neuroanatomical abnormalities, metabolic alterations, and obesity.

Conclusions:

  • The BBSome plays a critical role in modulating mitochondrial function.
  • Mitochondrial dysfunction is a key disease mechanism in Bardet-Biedl syndrome.
  • Targeting DRP1 activity offers a potential therapeutic strategy for BBS.