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

ATP Synthase: Mechanism01:48

ATP Synthase: Mechanism

18.9K
In animals, the mitochondrial F1F0 ATP synthase is the key protein that synthesizes ATP molecules through a complex catalytic mechanism. While the nuclear genome encodes the majority of ATP synthase subunits, the mitochondrial genome encodes some of the enzyme's most critical components. The formation of this multi-subunit enzyme is a complex multi-step process regulated at the level of transcription, translation, and assembly. Defects in one or more of these steps can result in decreased...
18.9K

You might also read

Related Articles

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

Sort by
Same author

Shared and distinct lipid profiles in amygdala from sporadic and GBA-associated Parkinson's diseases.

NPJ Parkinson's disease·2026
Same author

SPROUTS_DB: An Implemented Database of Contaminants for Extracellular Vesicle Proteomics Studies.

Proteomics·2026
Same author

Alzheimer's Tau seeds-induced pathology enhances hippocampal extracellular diffusion.

Communications biology·2025
Same author

Phenotypic characterization of an Atp13a2 knockout rat model of Parkinson's disease.

NPJ Parkinson's disease·2025
Same author

Protocol for 3D Matrigel embedding of primary murine astrocytes for a physiologically relevant culture model.

STAR protocols·2025
Same author

Gliogenesis from the subventricular zone modulates the extracellular matrix at the glial scar after brain ischemia.

eLife·2025

Related Experiment Video

Updated: Apr 6, 2026

Modeling Charcot-Marie-Tooth Disease In Vitro by Transfecting Mouse Primary Motoneurons
07:43

Modeling Charcot-Marie-Tooth Disease In Vitro by Transfecting Mouse Primary Motoneurons

Published on: January 7, 2019

7.5K

Progress in modelling ATP13A2-linked neurodegeneration.

Benedetta Balbo1, Rémi Kinet2, Laura Civiero1,3

  • 1Department of Biology, University of Padova, Padova, Italy.

NPJ Parkinson'S Disease
|April 4, 2026
PubMed
Summary

The ATP13A2 protein is crucial for brain health, and its dysfunction causes Parkinsonism. Research models are advancing our understanding of ATP13A2

More Related Videos

Modeling Mitochondrial Disease Using Brain Organoids: A Focus on Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes
08:56

Modeling Mitochondrial Disease Using Brain Organoids: A Focus on Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes

Published on: October 10, 2025

887
Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis
08:59

Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis

Published on: July 16, 2021

3.2K

Related Experiment Videos

Last Updated: Apr 6, 2026

Modeling Charcot-Marie-Tooth Disease In Vitro by Transfecting Mouse Primary Motoneurons
07:43

Modeling Charcot-Marie-Tooth Disease In Vitro by Transfecting Mouse Primary Motoneurons

Published on: January 7, 2019

7.5K
Modeling Mitochondrial Disease Using Brain Organoids: A Focus on Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes
08:56

Modeling Mitochondrial Disease Using Brain Organoids: A Focus on Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes

Published on: October 10, 2025

887
Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis
08:59

Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis

Published on: July 16, 2021

3.2K

Area of Science:

  • Neuroscience
  • Genetics
  • Biochemistry

Background:

  • ATP13A2 is a lysosomal P5-ATPase vital for the central nervous system.
  • It plays a key role in maintaining homeostasis of polyamines, metal cations, and calcium.
  • Mutations in ATP13A2 lead to Kufor-Rakeb syndrome, a juvenile Parkinson's disease.

Purpose of the Study:

  • To review the current understanding of ATP13A2 structure, function, and pathology.
  • To summarize insights gained from various cellular and animal models of ATP13A2 dysfunction.
  • To highlight the utility of these models in neurodegeneration research and therapeutic development.

Main Methods:

  • Literature review of studies on ATP13A2.
  • Analysis of data from cellular models.
  • Examination of findings from animal models.

Main Results:

  • Numerous ATP13A2-related models have been developed since the discovery of Kufor-Rakeb syndrome.
  • These models have significantly advanced the understanding of ATP13A2's physiological and pathophysiological roles.
  • Insights from these models are crucial for elucidating disease mechanisms.

Conclusions:

  • ATP13A2 research, particularly utilizing diverse models, is essential for understanding neurodegenerative diseases.
  • These models offer valuable platforms for developing targeted therapies.
  • Further research across species and experimental systems will broadly benefit neurodegeneration studies.