Search research articles

ABOUT JoVE

Overview Leadership Blog JoVE Help Center

AUTHORS

Publishing Process Editorial Board Scope & Policies Peer Review FAQ Submit

LIBRARIANS

Testimonials Subscriptions Access Resources Library Advisory Board FAQ

RESEARCH

JoVE Journal Methods Collections JoVE Encyclopedia of Experiments Archive

EDUCATION

JoVE Core JoVE Business JoVE Science Education JoVE Lab Manual Faculty Resource Center Faculty Site

Terms & Conditions of Use

Related Concept Videos

Mitochondrial Membranes

Mitochondrial Membranes

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,...

The Inner Mitochondrial Membrane

The Inner Mitochondrial Membrane

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...

Translocation of Proteins into the Mitochondria

Translocation of Proteins into the Mitochondria

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,...

Mitochondrial Protein Sorting

Mitochondrial Protein Sorting

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...

Porin Insertion in the Outer Mitochondrial Membrane

Porin Insertion in the Outer Mitochondrial Membrane

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...

Structure of Porins

Structure of Porins

Mitochondria, chloroplasts, and gram-negative bacteria have transmembrane, beta-barrel proteins called porins to mediate the free diffusion of ions and metabolites across the membrane. Mitochondrial porin precursors contain conserved amino acid sequences called beta signals at their C-terminal. Beta signals have a motif of PoXGXXHyXHy (Po-Polar, X-Any amino acid, G-Glycine, Hy-LargeHydrophobic), which are crucial for precursor recognition to initiate precursor assembly. Beta-barrel...

You might also read

Related Articles

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

Sort by

Same author

Delayed molecular aging, preservation of energy metabolism and enhanced exercise response in exercise-trained human muscle.

Nature aging·2026

Same author

DHDDS-related juvenile parkinsonism is caused by impaired lipid metabolism, glycosylation, and mitochondrial dysfunction, which can be rescued by NAD⁺ treatment.

medRxiv : the preprint server for health sciences·2026

Same author

Diagnostic Criteria and Management of MELAS and Stroke-Like Episodes: Consensus-Based Statements.

European journal of neurology·2026

Same author

Pilot Study of Fingolimod Treatment in Neuronal Ceroid Lipofuscinosis Type 1.

Neurology. Genetics·2026

Same author

Liver Involvement in POLG Disease-a Multicentre Cohort Study of 202 Patients.

Journal of inherited metabolic disease·2025

Same author

Palliative Care for Children and Adults With Inherited Metabolic Disease in Europe: An Underutilised Service for Supportive Treatment and Care.

Journal of inherited metabolic disease·2025

Search research articles

Related Experiment Video

Updated: Jun 24, 2025

A Model Membrane Platform for Reconstituting Mitochondrial Membrane Dynamics

A Model Membrane Platform for Reconstituting Mitochondrial Membrane Dynamics

Published on: September 2, 2020

Mitochondrial membrane synthesis, remodelling and cellular trafficking.

Martina Messina^1,2, Frédéric M Vaz^3,4, Shamima Rahman^1,2

¹Mitochondrial Research Group, Genetics and Genomic Medicine Department, UCL Great Ormond Street Institute of Child Health, London, UK.

Journal of Inherited Metabolic Disease

|June 14, 2024

Summary

Mitochondrial disorders linked to lipid synthesis and membrane issues cause diverse symptoms. Understanding these complex interactions is key to addressing cellular dysfunction and improving human health.

Keywords:

MAM MERC MICOS cardiolipin cell trafficking mitochondrial lipid biosynthesis organellar crosstalk primary mitochondrial disease

More Related Videos

Author Spotlight: Decoding Mitochondrial Aging

Author Spotlight: Decoding Mitochondrial Aging

Published on: June 30, 2023

Mitochondria-associated ER Membranes MAMs and Glycosphingolipid Enriched Microdomains GEMs: Isolation from Mouse Brain

Mitochondria-associated ER Membranes MAMs and Glycosphingolipid Enriched Microdomains GEMs: Isolation from Mouse Brain

Published on: March 4, 2013

Related Experiment Videos

Last Updated: Jun 24, 2025

A Model Membrane Platform for Reconstituting Mitochondrial Membrane Dynamics

A Model Membrane Platform for Reconstituting Mitochondrial Membrane Dynamics

Published on: September 2, 2020

Author Spotlight: Decoding Mitochondrial Aging

Author Spotlight: Decoding Mitochondrial Aging

Published on: June 30, 2023

Mitochondria-associated ER Membranes MAMs and Glycosphingolipid Enriched Microdomains GEMs: Isolation from Mouse Brain

Mitochondria-associated ER Membranes MAMs and Glycosphingolipid Enriched Microdomains GEMs: Isolation from Mouse Brain

Published on: March 4, 2013

Area of Science:

Cell Biology
Genetics
Biochemistry

Background:

Mitochondria are vital organelles involved in metabolism and signaling.
Primary mitochondrial disorders are genetic conditions affecting mitochondrial structure and function.
Emerging disorders involve defects in mitochondrial lipid biosynthesis, particularly cardiolipin, and membrane biology.

Purpose of the Study:

To review lipid synthesis in mitochondrial membranes.
To discuss the mitochondrial contact site and cristae organizing system (MICOS).
To explore mitochondrial dynamics, trafficking, and associated disorders.

Main Methods:

Literature review of mitochondrial lipid synthesis.
Analysis of MICOS, mitochondrial dynamics, and trafficking.
Examination of inter-organelle membrane interactions.

Main Results:

Defects in cardiolipin synthesis and mitochondrial membrane biology cause clinical heterogeneity with common features like cardiomyopathy and neurodegeneration.
Overlapping protein functions in lipid biosynthesis and import suggest coordinated mitochondrial processes.
Disrupted inter-organelle membrane interactions, particularly with the ER, contribute to secondary mitochondrial dysfunction.

Conclusions:

Dysregulation of mitochondrial lipid synthesis, membrane organization, and dynamics underlies a spectrum of human diseases.
Inter-organelle communication is crucial for mitochondrial function and overall cellular homeostasis.
Further research into these interconnected processes is essential for understanding and treating mitochondrial disorders.