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Elevated plasma complement components in facioscapulohumeral dystrophy.

Chao-Jen Wong1, Leo Wang2, V Michael Holers3

  • 1Division of Human Biology, Fred Hutchinson Cancer Research Center, Seattle, WA 98109, USA.

Human Molecular Genetics
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Facioscapulohumeral dystrophy (FSHD) involves elevated complement system activation. Measuring complement factors in plasma may serve as a non-invasive biomarker for FSHD disease activity and treatment response.

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Area of Science:

  • Biochemistry
  • Immunology
  • Neurology

Background:

  • Facioscapulohumeral dystrophy (FSHD) pathophysiology understanding is advancing.
  • Therapeutic approaches for FSHD are progressing into clinical trials.
  • Biomarkers for FSHD disease activity and progression are needed.

Purpose of the Study:

  • To investigate complement system activation in FSHD patients.
  • To identify plasma-based biomarkers for FSHD.
  • To assess the potential of complement activation as a non-invasive FSHD biomarker.

Main Methods:

  • Plasma samples from two independent cohorts of FSHD and control subjects were analyzed.
  • Complement components in plasma were quantified.
  • Statistical analyses were performed to identify distinguishing factors.

Main Results:

  • Elevated complement components were detected in plasma from both FSHD cohorts.
  • Specific complement factors were identified that differentiate FSHD patients from controls.
  • A subset of FSHD patients exhibited elevated levels of multiple complement components.

Conclusions:

  • Complement activation is a feature of FSHD.
  • Plasma complement measurements show potential as non-invasive biomarkers for FSHD activity, progression, and therapy response.
  • Targeting the complement pathway may be a viable therapeutic strategy for FSHD.