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Impaired Muscle Mitochondrial Function in Familial Partial Lipodystrophy.

Vinaya Simha1, Ian R Lanza1, Surendra Dasari2

  • 1Divisions of Endocrinology, Mayo Clinic, Rochester, MN 55905, USA.

The Journal of Clinical Endocrinology and Metabolism
|October 6, 2021
PubMed
Summary

Familial partial lipodystrophy (FPL) causes increased muscle size but not strength, with earlier fatigue. Impaired mitochondrial function and altered gene expression contribute to muscle dysfunction in FPL.

Keywords:
insulin resistancelipodystrophymitochondriaskeletal muscle hypertrophy

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Area of Science:

  • Endocrinology
  • Metabolic Disorders
  • Skeletal Muscle Physiology

Background:

  • Familial partial lipodystrophy (FPL), Dunnigan variety, is characterized by skeletal muscle hypertrophy and insulin resistance.
  • The underlying causes and functional consequences of muscle hypertrophy in FPL remain unclear.

Purpose of the Study:

  • To compare muscle strength, endurance, and protein synthesis rates between FPL subjects and matched controls.
  • To investigate skeletal muscle mitochondrial function and gene expression patterns in FPL.

Main Methods:

  • Dual-energy X-ray absorptiometry for body composition and minimal modeling for insulin sensitivity.
  • Assessment of muscle strength, endurance, and skeletal muscle biopsy for protein synthesis.
  • Mitochondrial respirometry, transcriptome, proteome, and gene set enrichment analysis were performed.

Main Results:

  • FPL subjects exhibited increased muscularity but not strength, with earlier fatigue.
  • Mitochondrial function was impaired, indicated by decreased respiration with fatty acid substrate, elevated lactate, and reduced acylcarnitine.
  • Gene expression analysis revealed downregulation of metabolic pathways, accelerated aging patterns, and reduced muscle protein synthesis.

Conclusions:

  • Increased muscularity in FPL is likely due to reduced protein degradation, not increased synthesis.
  • Impaired mitochondrial function and altered gene expression are key factors in FPL-associated metabolic abnormalities and skeletal muscle dysfunction.