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

Translocation of Proteins into the Mitochondria01:19

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 Sorting01:39

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...
Energy to Drive Translocation01:37

Energy to Drive Translocation

Mitochondrial protein import is powered by two distinct energy sources: ATP hydrolysis and electrochemical potential across the inner membrane. Newly synthesized precursors are bound by cytosolic chaperones of the Hsp70 family, which guide them to the import receptors on the mitochondrial surface. Utilizing the energy of ATP hydrolysis, Hsp70 chaperones transfer these precursors to the TOM receptors on the mitochondrial outer membrane.
Generally, polypeptides are unfolded by two distinct...
Protein Transport into the Inner Mitochondrial Membrane01:34

Protein Transport into the Inner Mitochondrial Membrane

Nuclear encoded mitochondrial precursors are imported to the inner membrane in a multistep process involving two separate translocons, TIM22 and TIM23. TIM23 is a cation-selective pore that remains closed by the N terminal segment of the protein. Negative charges on the TIM23 act as a receptor for the incoming precursor, pulling the positively charged matrix-targeting sequence for peptide insertion and translocation.
Transport of mitochondrial precursors across the TIM23 channel is driven by...
Mitochondrial Membranes01:45

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,...
Mitochondrial Membranes01:45

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,...

You might also read

Related Articles

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

Sort by
Same author

Kinetochore proteins control microtubule dynamics in postmitotic neurons to regulate the formation of dendritic spines.

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

Energy stress activates AMPK to arrest mitochondria via phosphorylation of TRAK1.

The Journal of cell biology·2026
Same author

Miro GTPase domains regulate the assembly of the mitochondrial motor-adaptor complex.

Life science alliance·2022
Same author

Virtual screening for small-molecule pathway regulators by image-profile matching.

Cell systems·2022
Same author

Mitochondrial hitch-hiking of <i>Pink1</i> mRNA supports axonal mitophagy.

Autophagy·2022
Same author

Serine/Threonine Protein Phosphatase 2A Regulates the Transport of Axonal Mitochondria.

Frontiers in cellular neuroscience·2022
Same journal

Evolutionary and Biochemical Perspectives on the Incorporation and Utilization of Selenocysteine.

Cold Spring Harbor perspectives in biology·2026
Same journal

The Mitochondrial Calcium Uniporter: From Parts to Signaling Networks.

Cold Spring Harbor perspectives in biology·2026
Same journal

Growth Control and Beyond: Functional Diversity and Regulation of the Hippo Pathway in the Nervous System.

Cold Spring Harbor perspectives in biology·2026
Same journal

Structural Studies of Core Hippo Pathway Components.

Cold Spring Harbor perspectives in biology·2026
Same journal

The Hippo Pathway in Intestinal Regeneration, Fetal Reprogramming, and Tumorigenesis.

Cold Spring Harbor perspectives in biology·2026
Same journal

A Synergy between Genetics and Biochemistry Unravels the Molecular Architecture of the Hippo Signaling Pathway.

Cold Spring Harbor perspectives in biology·2026
See all related articles

Related Experiment Video

Updated: May 10, 2026

Three-dimensional Imaging and Analysis of Mitochondria within Human Intraepidermal Nerve Fibers
10:31

Three-dimensional Imaging and Analysis of Mitochondria within Human Intraepidermal Nerve Fibers

Published on: September 29, 2017

Mitochondrial trafficking in neurons.

Thomas L Schwarz1

  • 1F.M. Kirby Neurobiology Center, Children's Hospital Boston, and Department of Neurobiology, Harvard Medical School, Boston, Massachusetts 02115, USA. Thomas.schwarz@childrens.harvard.edu

Cold Spring Harbor Perspectives in Biology
|June 5, 2013
PubMed
Summary
This summary is machine-generated.

Neurons rely on mitochondrial trafficking, a process involving Miro and Milton proteins, for survival. This motor/adaptor complex regulates mitochondrial movement, crucial for cellular energy supply and maintenance.

More Related Videos

Analyzing Mitochondrial Transport and Morphology in Human Induced Pluripotent Stem Cell-Derived Neurons in Hereditary Spastic Paraplegia
07:32

Analyzing Mitochondrial Transport and Morphology in Human Induced Pluripotent Stem Cell-Derived Neurons in Hereditary Spastic Paraplegia

Published on: February 9, 2020

Neuromodulation and Mitochondrial Transport: Live Imaging in Hippocampal Neurons over Long Durations
04:50

Neuromodulation and Mitochondrial Transport: Live Imaging in Hippocampal Neurons over Long Durations

Published on: June 17, 2011

Related Experiment Videos

Last Updated: May 10, 2026

Three-dimensional Imaging and Analysis of Mitochondria within Human Intraepidermal Nerve Fibers
10:31

Three-dimensional Imaging and Analysis of Mitochondria within Human Intraepidermal Nerve Fibers

Published on: September 29, 2017

Analyzing Mitochondrial Transport and Morphology in Human Induced Pluripotent Stem Cell-Derived Neurons in Hereditary Spastic Paraplegia
07:32

Analyzing Mitochondrial Transport and Morphology in Human Induced Pluripotent Stem Cell-Derived Neurons in Hereditary Spastic Paraplegia

Published on: February 9, 2020

Neuromodulation and Mitochondrial Transport: Live Imaging in Hippocampal Neurons over Long Durations
04:50

Neuromodulation and Mitochondrial Transport: Live Imaging in Hippocampal Neurons over Long Durations

Published on: June 17, 2011

Area of Science:

  • Neuroscience
  • Cell Biology
  • Mitochondrial Dynamics

Background:

  • Neurons have a high energy demand, making mitochondrial trafficking essential for their survival and function.
  • Mitochondrial movement is critical for maintaining energy supply and demand balance within the complex neuronal architecture.
  • The polarized microtubule network in neuronal axons provides a unique system for studying mitochondrial motility.

Purpose of the Study:

  • To elucidate the motor/adaptor complex responsible for mitochondrial trafficking in neurons.
  • To understand the regulation of mitochondrial movement by intracellular signals.
  • To explore the role of mitochondrial trafficking in neuronal energy supply and organelle turnover.

Main Methods:

  • Biochemical assays in conjunction with studies of neuronal cell types.
  • Investigation of the motor/adaptor complex including kinesin, dynein, Miro (RhoT1/2), and Milton (TRAK1/2).
  • Utilizing the uniformly polarized microtubules in extended neuronal axons to study mitochondrial motility.

Main Results:

  • A shared motor/adaptor complex involving Miro and Milton proteins has been identified on the mitochondrial surface.
  • This complex, along with kinesin and dynein, mediates significant mitochondrial movement in neurons.
  • Understanding this complex provides insights into the regulation of mitochondrial trafficking by intracellular signals.

Conclusions:

  • Mitochondrial trafficking, mediated by the Miro-Milton complex, is vital for neuronal survival and function.
  • Regulation of mitochondrial movement is key to matching energy supply with demand and managing mitochondrial populations.
  • Neuronal axons serve as valuable models for dissecting the mechanisms of mitochondrial motility.