Jove
Visualize
Contáctanos
JoVE
x logofacebook logolinkedin logoyoutube logo
ACERCA DE JoVE
Visión GeneralLiderazgoBlogCentro de Ayuda JoVE
AUTORES
Proceso de PublicaciónConsejo EditorialAlcance y PolíticasRevisión por ParesPreguntas FrecuentesEnviar
BIBLIOTECARIOS
TestimoniosSuscripcionesAccesoRecursosConsejo Asesor de BibliotecasPreguntas Frecuentes
INVESTIGACIÓN
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchivo
EDUCACIÓN
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualCentro de Recursos para ProfesoresSitio de Profesores
Términos y Condiciones de Uso
Política de Privacidad
Políticas

Videos de Conceptos Relacionados

Amyloid Fibrils03:03

Amyloid Fibrils

11.9K
Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining,...
11.9K
Amyloid Fibrils03:03

Amyloid Fibrils

6.4K
6.4K
Alternative RNA Splicing02:18

Alternative RNA Splicing

25.2K
Alternative RNA splicing is the regulated splicing of exons and introns to produce different mature mRNAs from a single pre-mRNA. Unlike in constitutive splicing where a single gene produces a single type of mRNA, alternative splicing allows an organism to produce multiple proteins from a single gene and plays an important role in protein diversity.
There are five types of alternative RNA splicing that vary in the ways the pre-mRNA segments are removed or retained in the mature mRNA. The first...
25.2K
RNA Editing02:23

RNA Editing

9.9K
RNA editing is a post-transcriptional modification where a precursor mRNA (pre-mRNA) nucleotide sequence is changed by base insertion, deletion, or modification. The extent of RNA editing varies from a few hundred bases, in mitochondrial DNA of trypanosomes, to a just single base, in nuclear genes of mammals. Even a single base change in the pre-mRNA can convert a codon for one amino acid into the codon for another amino acid or a stop codon. This type of re-coding can significantly affect the...
9.9K
Bacterial RNA Polymerase00:43

Bacterial RNA Polymerase

32.8K
Unlike eukaryotes, bacteria use a single RNA Polymerase (RNAP) to transcribe all genes. The different subunits of bacterial RNAPhave distinct functions. The multisubunit structure of the bacterial RNAP helps the enzyme to maintain catalytic function, facilitate assembly, interact with DNA and RNA, and self-regulate its activity.
In most genes, the transcription site is a single base present upstream of the coding sequence. Though RNAP is a catalytically efficient enzyme, it does not recognize...
32.8K
Eukaryotic RNA Polymerases00:58

Eukaryotic RNA Polymerases

27.1K
RNA Polymerase (RNAP) is conserved in all animals, with bacterial, archaeal, and eukaryotic RNAPs sharing significant sequence, structural, and functional similarities. Among the three eukaryotic RNAPs, RNA Polymerase II is most similar to bacterial RNAP in terms of both structural organization and folding topologies of the enzyme subunits. However, these similarities are not reflected in their mechanism of action.
All three eukaryotic RNAPs require specific transcription factors, of which the...
27.1K

También podría leer

Artículos Relacionados

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

Ordenar por
Same author

Chemoradiation (CCRT) Effects on Head and Neck Muscles: Insights Into CCRT-Induced Dysphagia.

The Laryngoscope·2026
Same author

Physical interactions within the SIR heterochromatin complex potentiate inter-subunit communication and gene repression.

Cell reports·2026
Same author

A mutation in the nuclear speckle and splicing factor SRRM2 is associated with multisystem proteinopathy and causes dysregulation of synapse-associated genes.

RNA (New York, N.Y.)·2026
Same author

The assembly of stress granules during foot-and-mouth disease virus infection is uncoupled from activation of cellular intrinsic antiviral signalling.

PLoS pathogens·2026
Same author

TDP-43 Sustains Satellite Cells to Maintain and Regenerate Skeletal Muscle.

bioRxiv : the preprint server for biology·2026
Same author

Alphaviral non-structural protein-host RBP co-condensation as a mechanism to sustain virus replication.

Molecular cell·2026

Video Experimental Relacionado

Updated: Feb 2, 2026

Measuring Glucose Uptake in Drosophila Models of TDP-43 Proteinopathy
07:07

Measuring Glucose Uptake in Drosophila Models of TDP-43 Proteinopathy

Published on: August 3, 2021

3.2K

El TDP-43 y el ARN forman miogranulos similares al amiloide en el músculo regenerado

Thomas O Vogler1,2, Joshua R Wheeler2,3, Eric D Nguyen2,4

  • 1Department of Molecular, Cellular and Developmental Biology, University of Colorado, Boulder, CO, USA.

Nature
|November 23, 2018
PubMed
Resumen

Los agregados citoplasmáticos de la proteína TDP-43 son comunes en las enfermedades neuromusculares. Los investigadores descubrieron que la regeneración muscular normal implica que el TDP-43 forme "myo-granulas" temporales, que pueden conducir a agregados de enfermedad si no se eliminan.

Más Videos Relacionados

Optogenetic Phase Transition of TDP-43 in Spinal Motor Neurons of Zebrafish Larvae
07:14

Optogenetic Phase Transition of TDP-43 in Spinal Motor Neurons of Zebrafish Larvae

Published on: February 25, 2022

6.6K
Myo-mechanical Analysis of Isolated Skeletal Muscle
08:42

Myo-mechanical Analysis of Isolated Skeletal Muscle

Published on: February 22, 2011

27.6K

Videos de Experimentos Relacionados

Last Updated: Feb 2, 2026

Measuring Glucose Uptake in Drosophila Models of TDP-43 Proteinopathy
07:07

Measuring Glucose Uptake in Drosophila Models of TDP-43 Proteinopathy

Published on: August 3, 2021

3.2K
Optogenetic Phase Transition of TDP-43 in Spinal Motor Neurons of Zebrafish Larvae
07:14

Optogenetic Phase Transition of TDP-43 in Spinal Motor Neurons of Zebrafish Larvae

Published on: February 25, 2022

6.6K
Myo-mechanical Analysis of Isolated Skeletal Muscle
08:42

Myo-mechanical Analysis of Isolated Skeletal Muscle

Published on: February 22, 2011

27.6K

Área de la Ciencia:

  • La bioquímica
  • Biología molecular
  • La neurociencia

Sus antecedentes:

  • La agregación citoplasmática de TDP-43 es una característica de las enfermedades neuromusculares como la ELA y la miopatía de cuerpo de inclusión.
  • Si bien las mutaciones de TARDBP causan cierta agregación de TDP-43, la mayoría de los pacientes tienen agregados de tipo salvaje de TDP-43, lo que sugiere un mecanismo desconocido.

Objetivo del estudio:

  • Investigar el mecanismo detrás de la agregación de TDP-43 de tipo salvaje en enfermedades neuromusculares.
  • Explorar la función normal del TDP-43 en el músculo esquelético y su papel potencial en la patogénesis de la enfermedad.

Principales métodos:

  • Estudió el papel de TDP-43 en la regeneración del músculo esquelético en modelos de ratón y humanos.
  • Los ensamblajes citoplasmáticos caracterizados que contienen TDP-43 (myo-granulas).
  • Se evaluó la formación de miogranulos, la unión al ARNm, el aclaramiento y el potencial para la siembra de fibrillas amiloides.

Principales resultados:

  • El TDP-43 es esencial para la formación del músculo esquelético y forma "myo-granules" transitorios similares a los amiloides durante la regeneración.
  • Estos miogranulos se unen a los ARNm para las proteínas sarcoméricas y se eliminan a medida que el músculo madura.
  • Los mio-granulos pueden sembrar fibrillas amiloides TDP-43 in vitro y están elevados en un modelo de ratón con miopatía de cuerpo de inclusión.

Conclusiones:

  • Las miogranulas representan un conjunto normal y transitorio de TDP-43 durante la regeneración muscular.
  • La desregulación en el ensamblaje o el aclaramiento de los miogranulos puede ser una fuente primaria de agregados tóxicos de TDP-43 en enfermedades neuromusculares comunes.