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Related Concept Videos

Translation01:31

Translation

17.0K
Translation is the process of synthesizing proteins from the genetic information carried by messenger RNA (mRNA). Following transcription, it constitutes the final step in the expression of genes. This process is carried out by ribosomes, complexes of protein and specialized RNA molecules. Ribosomes, transfer RNA (tRNA), and other proteins produce a chain of amino acids—the polypeptide—as the end product of translation.
Translation Produces the Building Blocks of Life
Proteins are...
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Translation01:31

Translation

153.2K
Lesson: Translation
Translation is the process of synthesizing proteins from the genetic information carried by messenger RNA (mRNA). Following transcription, it constitutes the final step in the expression of genes. This process is carried out by ribosomes, complexes of protein and specialized RNA molecules. Ribosomes, transfer RNA (tRNA), and other proteins produce a chain of amino acids—the polypeptide—as the end product of translation.
Translation Produces the Building Blocks of...
153.2K
Translocation of Proteins into the Mitochondria01:19

Translocation of Proteins into the Mitochondria

10.3K
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,...
10.3K
Porin Insertion in the Outer Mitochondrial Membrane01:12

Porin Insertion in the Outer Mitochondrial Membrane

4.0K
Porins are beta-barrel proteins translocated to the mitochondrial outer membrane through the TOM complex into the intermembrane space. Porin precursors bind TIM chaperones within the intermembrane space and are guided to the Sorting and Assembly Machinery complex or SAM complex on the outer mitochondrial membrane.
Three models describe the assembly of porins by the SAM complex and their insertion into the outer membrane. Model 1 suggests that porins are assembled outside the SAM channel as the...
4.0K
ATP Synthase: Mechanism01:48

ATP Synthase: Mechanism

16.0K
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...
16.0K
Export of Misfolded Proteins out of the ER01:32

Export of Misfolded Proteins out of the ER

4.7K
After folding, the ER assesses the quality of secretory and membrane proteins. The correctly folded proteins are cleared by the calnexin cycle for transport to their final destination, while misfolded proteins are held back in the ER lumen. The ER chaperones attempt to unfold and refold the misfolded proteins but sometimes fail to achieve the correct native conformation. Such terminally misfolded proteins are then exported to the cytosol by ER-associated degradation or ERAD pathway for...
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Related Experiment Video

Updated: Nov 26, 2025

Rapid Isolation of the Mitoribosome from HEK Cells
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Rapid Isolation of the Mitoribosome from HEK Cells

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The Diseased Mitoribosome.

Alberto Ferrari1, Samuel Del'Olio1,2, Antoni Barrientos1,3

  • 1Department of Neurology, University of Miami Miller School of Medicine, FL, USA.

FEBS Letters
|December 14, 2020
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Summary
This summary is machine-generated.

Mitochondria

Keywords:
OXPHOS deficiencymitochondrial diseasemitochondrial ribosomemitochondrial translationmitoribosome assembly

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Area of Science:

  • Cell Biology
  • Genetics
  • Biochemistry

Background:

  • Mitochondria possess a unique genome and protein synthesis machinery called the mitoribosome.
  • Mitoribosome biogenesis requires nuclear and mitochondrial genetic elements.
  • Recent cryo-electron microscopy (cryo-EM) studies reveal mitoribosome structure and function.

Purpose of the Study:

  • To review current knowledge of mitoribosome assembly and function.
  • To explore the clinical aspects of mitochondrial disorders linked to mitoribosome defects.
  • To highlight the synergy between basic research and clinical studies in mitochondrial medicine.

Main Methods:

  • Literature review of fundamental mitoribosome research.
  • Analysis of clinical data on mitochondrial disorders.
  • Integration of structural biology findings (cryo-EM) with genetic and clinical information.

Main Results:

  • Mitoribosome dysfunction due to mutations causes severe inherited mitochondrial disorders.
  • Defects in mitoribosome biogenesis lead to protein synthesis impairment and respiratory chain failure.
  • Specific disorders include encephalomyocardiopathy, deafness, neuropathy, and developmental delays.

Conclusions:

  • Understanding mitoribosome assembly and function is crucial for diagnosing and treating mitochondrial diseases.
  • Mutations in mitoribosome components provide insights into mitochondrial biology and disease mechanisms.
  • Combined basic and clinical research advances both mitochondrial medicine and fundamental biology.