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Videos de Conceptos Relacionados

ABC Transporters: Importer01:27

ABC Transporters: Importer

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ATP-binding cassette or ABC transporters are a class of ATP-driven pumps that hydrolyze ATP to move solutes across the membrane. They can be grouped into importers and exporters. While exporters are present in all domains of life, importers exist only in bacteria and some plants.
In bacteria, based on the number of transmembrane helices and the chemical nature of their substrates, the ABC importers can be divided into three types:
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Cotranslational Protein Translocation01:20

Cotranslational Protein Translocation

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Translocation of proteins across membranes is an ancient process that occurs even in bacteria and archaebacteria. In fact, the components of the translocation machinery are still conserved between prokaryotes and eukaryotes.
Sec61 channel partners for cotranslational translocation
During cotranslational translocation, the Sec61 channel partners with the signal recognition particle (SRP), the signal recognition particle receptor (SR), and the ribosomes to transport the nascent polypeptide chain...
8.3K
Insertion of Single-pass Transmembrane Proteins in the RER01:26

Insertion of Single-pass Transmembrane Proteins in the RER

13.0K
Integral membrane proteins are proteins adhered to the lipid bilayer of a cell organelle or membrane. They can be of two types: transmembrane integral proteins that span the lipid bilayer and monotopic proteins that are attached to either side of the membrane but do not pass through it.
Integral transmembrane proteins possess transmembrane and extra membrane domains. The transmembrane domains are primarily made of 20-25 hydrophobic amino acids arranged in a helical secondary confirmation. These...
13.0K
Total Internal Reflection Fluorescence Microscopy01:05

Total Internal Reflection Fluorescence Microscopy

11.0K
Total internal reflection fluorescence microscopy or TIRF is an advanced microscopic technique used to visualize fluorophores in samples close to a solid surface with a higher refractive index, such as a glass coverslip. TIRF only allows fluorophores in proximity to the solid surface to be excited. When light from a medium with a lower refractive index (such as air) hits the glass coverslip at a critical angle, the light undergoes total internal reflection stead of passing through the glass.
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Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

1.1K
Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
1.1K
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

657
Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
657

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Native CFTR codon bias controls translation rate to balance off-pathway aggregation and channel function by conformational imprinting.

Scientific reports·2025
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In vitro modulator responsiveness of 655 CFTR variants found in people with cystic fibrosis.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society·2024
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A small molecule high throughput screening platform to profile conformational properties of nascent, ribosome-bound proteins.

Scientific reports·2022
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CFTR trafficking mutations disrupt cotranslational protein folding by targeting biosynthetic intermediates.

Nature communications·2020
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Towards next generation therapies for cystic fibrosis: Folding, function and pharmacology of CFTR.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society·2020
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Engineered transfer RNAs for suppression of premature termination codons.

Nature communications·2019

Video Experimental Relacionado

Updated: May 2, 2026

Expression and Purification of the Cystic Fibrosis Transmembrane Conductance Regulator Protein in Saccharomyces cerevisiae
14:56

Expression and Purification of the Cystic Fibrosis Transmembrane Conductance Regulator Protein in Saccharomyces cerevisiae

Published on: March 10, 2012

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CFTR: nuevos miembros se unen al redil.

William R Skach1

  • 1Department of Biochemistry and Molecular Biology, Oregon Health Sciences University, Portland, OR 97239, USA. skachw@ohsu.edu

Cell
|November 18, 2006
PubMed
Resumen

Los investigadores identificaron Aha1, un nuevo chaperón Hsp90, crucial para el plegamiento de la proteína reguladora de conductividad transmembrana de la fibrosis quística (CFTR). Este hallazgo arroja luz sobre las vías de plegamiento y degradación de la CFTR en la fibrosis quística.

Área de la Ciencia:

  • Biología Molecular Biología Molecular
  • Biología celular Biología celular.
  • El plegamiento de las proteínas.

Sus antecedentes:

  • La homeostasis de las proteínas de la membrana implica procesos celulares complejos que controlan el plegamiento, el mal plegamiento y la degradación.
  • La fibrosis quística se caracteriza por el mal plegamiento y la degradación prematura del canal de cloruro del regulador de conductividad transmembrana (CFTR) de la fibrosis quística.
  • Comprender el procesamiento de la CFTR es fundamental para desarrollar estrategias terapéuticas.

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