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Metabolic reprogramming in the OPA1-deficient cells.

Wenting Dai1, Zhichao Wang1, Qiong A Wang1,2

  • 1Department of Molecular and Cellular Endocrinology, Arthur Riggs Diabetes and Metabolism Research Institute, City of Hope National Medical Center, Duarte, CA, USA.

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Optic Atrophy 1 (OPA1) deficiency impairs mitochondrial fusion and alters cell metabolism. OPA1-deficient cells activate glutamine metabolism for fatty acid synthesis and proliferation when glucose oxidation fails.

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Cell growthCitrateDe novo lipogenesisOPA1 dysfunctionOxidative metabolismReductive carboxylation

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Area of Science:

  • Mitochondrial biology
  • Cellular metabolism
  • Biochemistry

Background:

  • Optic Atrophy 1 (OPA1) is crucial for inner mitochondrial membrane fusion and is implicated in autosomal dominant optic atrophy.
  • OPA1 deficiency causes mitochondrial morphology defects, but its impact on central carbon metabolism is not fully understood.
  • Understanding OPA1's role in metabolism is key to exploring its function beyond mitochondrial dynamics.

Purpose of the Study:

  • To investigate the functional role of OPA1 in cellular metabolism beyond its known role in mitochondrial fusion.
  • To elucidate the metabolic mechanisms by which OPA1 deficiency affects cell fitness.
  • To explore OPA1's impact on central carbon metabolism and its consequences for cell proliferation and lipid synthesis.

Main Methods:

  • Utilized [U-13C]glucose and [U-13C]glutamine isotope tracing in OPA1-knockout (OPA1-KO) and wild-type (OPA1-WT) mouse embryonic fibroblasts (MEFs).
  • Applied metabolic flux analysis to integrate tracing data and understand metabolic reprogramming in OPA1-deficient cells.
  • Investigated the effect of inhibiting isocitrate dehydrogenase 1 (IDH1) using GSK321 on lipid synthesis and cell proliferation.

Main Results:

  • OPA1-deficient MEFs exhibited depleted intracellular citrate and reduced oxygen consumption rates, indicative of impaired mitochondrial function.
  • Oxidative glucose metabolism was compromised, while OPA1 deficiency induced glutamine-dependent reductive carboxylation.
  • This reductive metabolism served as the primary source of cytosolic citrate for de novo fatty acid synthesis, supporting cell proliferation.
  • Inhibition of IDH1 significantly repressed lipid synthesis and proliferation in OPA1-deficient MEFs.

Conclusions:

  • OPA1 deficiency reprograms cellular metabolism, shifting reliance from glucose oxidation to glutamine-dependent reductive carboxylation.
  • This metabolic adaptation supports lipogenesis and proliferation when oxidative glucose metabolism is insufficient due to impaired mitochondrial fusion.
  • OPA1 plays a critical role in maintaining metabolic homeostasis and cell fitness, particularly under conditions of mitochondrial dysfunction.