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Assessment of Submitochondrial Protein Localization in Budding Yeast Saccharomyces cerevisiae
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Mitochondrial complex III Rieske Fe-S protein processing and assembly.

Erika Fernandez-Vizarra1, Massimo Zeviani1

  • 1a MRC-Mitochondrial Biology Unit , University of Cambridge , Hills Road, CB2 0XY , Cambridge , UK.

Cell Cycle (Georgetown, Tex.)
|December 16, 2017
PubMed
Summary
This summary is machine-generated.

TTC19 deficiency causes mitochondrial disease by disrupting complex III assembly. This study reveals TTC19

Keywords:
Mitochondrial respiratory chainOXPHOSRieske Fe-S proteinTTC19UQCRFS1mitochondriamitochondrial complex IIImitochondrial disease

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Area of Science:

  • Mitochondrial biology
  • Molecular genetics
  • Biochemistry

Background:

  • Mitochondrial respiratory chain biogenesis is crucial for cellular energy production.
  • Mitochondrial diseases are linked to defects in the respiratory chain.
  • TTC19 is a recently identified gene associated with encephalopathy and mitochondrial complex III (cIII) deficiency.

Purpose of the Study:

  • To investigate the function of TTC19 in mitochondrial complex III (cIII) biogenesis.
  • To elucidate the role of TTC19 in the assembly and stability of the Rieske Fe-S protein (UQCRFS1).
  • To understand the implications of TTC19 deficiency for cIII activity and mitochondrial disease.

Main Methods:

  • Utilized TTC19-deficient human and mouse models.
  • Analyzed the assembly and processing of UQCRFS1 within cIII.
  • Assessed the impact of TTC19 deficiency on cIII enzymatic activity and overall function.

Main Results:

  • TTC19 deficiency leads to the accumulation of N-terminal fragments of UQCRFS1.
  • These fragments interfere with proper cIII assembly and function.
  • TTC19 acts as a post-assembly quality control factor for UQCRFS1.

Conclusions:

  • TTC19 plays a critical role in the 'husbandry' or quality control of UQCRFS1 during cIII biogenesis.
  • Dysfunctional UQCRFS1 processing due to TTC19 absence is detrimental to cIII activity.
  • Understanding TTC19 function offers insights into mitochondrial disease mechanisms and potential therapeutic targets.