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CCN6 regulates mitochondrial respiratory complex assembly and activity.

Deepesh Kumar Padhan1, Archya Sengupta1, Milan Patra2

  • 1Cancer Biology and Inflammatory Disorder Division, CSIR-Indian Institute of Chemical Biology, Kolkata, India.

FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology
|July 21, 2020
PubMed
Summary

Cellular communication network factor 6 (CCN6) regulates mitochondrial function and respiratory complex assembly. Mutations in CCN6 disrupt these processes, leading to mitochondrial defects and poor cell viability, contributing to Progressive Pseudo Rheumatoid Dysplasia (PPRD).

Keywords:
PPRDchondrocytemitochondria

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Area of Science:

  • Cellular Biology
  • Mitochondrial Biology
  • Musculoskeletal Disorders

Background:

  • Cellular communication network factor 6 (CCN6) mutations are associated with Progressive Pseudo Rheumatoid Dysplasia (PPRD).
  • The precise function of CCN6 and the pathogenesis of PPRD are not well understood.
  • Understanding CCN6's role is crucial for elucidating PPRD mechanisms.

Purpose of the Study:

  • To functionally characterize CCN6 and its disease-associated mutants.
  • To investigate the role of CCN6 in mitochondrial respiratory complex assembly and function.
  • To explore the impact of CCN6 mutations on chondrocyte viability and mitochondrial integrity.

Main Methods:

  • Size exclusion chromatography and native polyacrylamide gel electrophoresis to identify CCN6 complexation.
  • siRNA-mediated transfection and electron microscopy to assess effects on RER-mitochondria proximity.
  • CRISPR-Cas9 technology for generating CCN6 mutants.
  • Immunoblotting, Complex I activity assays, and MTT assays for functional analysis.

Main Results:

  • CCN6 was identified as a component of the mitochondrial respiratory complex in human chondrocytes.
  • Reduced CCN6 expression altered RER-mitochondria distance and enhanced mitochondrial respiratory complex assembly/activity.
  • PPRD-associated CCN6 mutants exhibited low expression, impaired respiratory complex function, abnormal mitochondria, and reduced cell viability.

Conclusions:

  • CCN6 regulates mitochondrial respiratory complex assembly and activity by modulating RER-mitochondria proximity.
  • CCN6 mutations disrupt mitochondrial function, leading to cellular defects and contributing to PPRD pathogenesis.
  • CCN6 is a key regulator of mitochondrial health, and its dysfunction has significant implications for musculoskeletal disorders.