Search research articles

ACERCA DE JoVE

Visión General Liderazgo Blog Centro de Ayuda JoVE

AUTORES

Proceso de Publicación Consejo Editorial Alcance y Políticas Revisión por Pares Preguntas Frecuentes Enviar

BIBLIOTECARIOS

Testimonios Suscripciones Acceso Recursos Consejo Asesor de Bibliotecas Preguntas Frecuentes

INVESTIGACIÓN

JoVE Journal Methods Collections JoVE Encyclopedia of Experiments Archivo

EDUCACIÓN

JoVE Core JoVE Business JoVE Science Education JoVE Lab Manual Centro de Recursos para Profesores Sitio de Profesores

Términos y Condiciones de Uso

Política de Privacidad

Videos de Conceptos Relacionados

Animal Mitochondrial Genetics

Animal Mitochondrial Genetics

Among all the organelles in an animal cell, only mitochondria have their own independent genomes. Animal mitochondrial DNA is a double-stranded, closed-circular molecule with around 20,000 base pairs. Mitochondrial DNA is unique in that one of its two strands, the heavy, or H, -strand is guanine rich, whereas the complementary strand is cytosine rich and called the light, or L, -strand. Compared to nuclear DNA, mitochondrial DNA has a very low percentage of non-coding regions and is marked by...

Mitochondria

Mitochondria

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,...

Mitochondrial Precursor Proteins

Mitochondrial Precursor Proteins

Mitochondrial precursors are partially unfolded or loosely folded polypeptide chains. Newly synthesized precursors are inhibited from spontaneously folding into their native conformation by the cytosolic chaperones, heat shock proteins 70 (Hsp70), and mitochondrial import stimulation factors (MSFs). Precursors bound to MSFs are guided to the TOM70-TOM37 receptors, while precursors bound to Hsp70 chaperones are targetted to TOM20-TOM22 receptor complexes.
Most of the mitochondrial...

Electron Transport Chain: Complex I and II

Electron Transport Chain: Complex I and II

The mitochondrial electron transport chain (ETC) is the main energy generation system in the eukaryotic cells. However, mitochondria also produce cytotoxic reactive oxygen species (ROS) due to the large electron flow during oxidative phosphorylation. While Complex I is one of the primary sources of superoxide radicals, ROS production by Complex II is uncommon and may only be observed in cancer cells with mutated complexes.
ROS generation is regulated and maintained at moderate levels necessary...

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,...

ATP Synthase: Mechanism

ATP Synthase: Mechanism

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...

También podría leer

Artículos Relacionados

Artículos vinculados a este trabajo por autores compartidos, revista y gráfico de citas.

Ordenar por

Same author

Patient Preferences in Neuromuscular Diseases: Insights for Future Drug Development.

JIMD reports·2026

Same author

Adjunctive transcranial DC stimulation in the management of refractory drug-resistant focal epilepsy in POLG mitochondrial disease: A case series.

Seizure·2026

Same author

Mavodelpar in patients with primary mitochondrial myopathy: a phase 1 trial.

Scientific reports·2026

Same author

Quantifying variability of mitochondrial markers in m3243A > G myopathy.

Scientific reports·2025

Same author

Elevated glioma-related cortical glutamate <i>in vivo</i> is associated with <i>ex vivo</i> interictal discharges in human brain slices.

Brain communications·2025

Same author

Model systems informing mechanisms and drug discovery: a review of <i>POLG</i>-related disease models.

Wellcome open research·2025

Search research articles

Video Experimental Relacionado

Updated: Nov 14, 2025

Author Spotlight: Decoding Mitochondrial Aging

Author Spotlight: Decoding Mitochondrial Aging

Published on: June 30, 2023

Enfermedades mitocondriales: esperanza para el futuro

Oliver M Russell¹, Gráinne S Gorman², Robert N Lightowlers³

¹Wellcome Centre for Mitochondrial Research, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne NE2 4HH, UK; Clinical and Translational Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne NE2 4HH, UK.

|March 30, 2020

Resumen

Las enfermedades mitocondriales provienen de mutaciones genéticas. Los tratamientos actuales manejan los síntomas, pero las nuevas terapias como las pequeñas moléculas, las terapias génicas y las intervenciones de la línea germinal tienen como objetivo corregir los defectos subyacentes.

Más Videos Relacionados

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model

Published on: March 9, 2022

Generation of Human Brain Organoids for Mitochondrial Disease Modeling

Generation of Human Brain Organoids for Mitochondrial Disease Modeling

Published on: June 21, 2021

Videos de Experimentos Relacionados

Last Updated: Nov 14, 2025

Author Spotlight: Decoding Mitochondrial Aging

Author Spotlight: Decoding Mitochondrial Aging

Published on: June 30, 2023

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model

Published on: March 9, 2022

Generation of Human Brain Organoids for Mitochondrial Disease Modeling

Generation of Human Brain Organoids for Mitochondrial Disease Modeling

Published on: June 21, 2021

Área de la Ciencia:

La genética
La bioquímica
Biología molecular

Sus antecedentes:

Las enfermedades mitocondriales son un grupo diverso de trastornos genéticos.
Los tratamientos actuales ofrecen principalmente un alivio sintomático, sin abordar la causa raíz.

Objetivo del estudio:

Revisar los avances recientes en el tratamiento de las enfermedades mitocondriales.
Explorar nuevas estrategias terapéuticas y medidas preventivas.

Principales métodos:

Revisión de la literatura sobre las terapias de moléculas pequeñas.
Análisis de los enfoques de la terapia génica.
Examen de las técnicas de prevención de la transmisión germinal.

Principales resultados:

Los fármacos emergentes de moléculas pequeñas son prometedores para tratar defectos específicos.
Las terapias génicas están avanzando para las mutaciones del ADN nuclear y mitocondrial.
Las intervenciones de la línea germinal ofrecen potencial para prevenir la herencia de enfermedades.

Conclusiones:

Se están logrando avances significativos en el desarrollo de tratamientos específicos para las enfermedades mitocondriales.
Una combinación de estrategias terapéuticas y métodos preventivos es crucial para el tratamiento de estos trastornos.