PIPages
Labs

Hongshuai Li

University of Iowa, IA·Physiology

Duchenne muscular dystrophy (DMD) is the most common muscular dystrophy in children. It is caused by loss of dystrophin, a protein that holds muscle fibers together.

Fields

Physiology

Medicine & Clinical Research

Pharmacology

Topics

Duchenne muscular dystrophy

dystrophin

utrophin

Grants
2 known grants, through Jun 2029

What they work on

  1. Identify which cells in dystrophic skeletal muscle produce the bone-regulating myokine FGF21 and the molecular signals that switch it on, using DMD mouse models.
  2. Determine how muscle-derived FGF21 affects bone, muscle and whole-body metabolism in DMD, using mice with FGF21 deleted only in skeletal muscle and cultured cell models.
  3. Test whether oral inorganic nitrite restores nitrate/nitrite levels, improves muscle and bone damage and preserves muscle function in dystrophin/utrophin double-knockout mice.
  4. Test whether nitrite acts on dystrophic muscle through myoglobin-driven NO-cGMP signaling, and protects bone by changing the bone-regulating myokines that muscle releases.

More about the research

The disease weakens muscle and also harms bone, leading to osteoporosis and fractures. No targeted therapies exist for these bone problems, and effective treatments for DMD as a whole remain limited. The lab studies how diseased muscle talks to bone through myokines, signaling molecules released by muscle. It works on two connected projects: one on a harmful myokine and one on a possible low-cost treatment.

Why it matters

Children with Duchenne muscular dystrophy face weak bones and fractures as well as muscle loss, and there are no targeted treatments for the bone disease. This work could identify FGF21 signaling as a drug target for bone health in DMD. It could also support oral nitrite as a safe, low-cost treatment for both muscle and bone. The findings may extend to other neuromuscular diseases with faulty nitric oxide signaling.

Known grants

From NIH and NSF research grants. Labs often have other funding we can't see.

Role of FGF21 in DMD

NIH · Aug 2024 – Jun 2029

Targeting Nitrate-Nitrite-NO pathway for Ameliorating Muscle and Bone Comorbidities in Duchenne Muscular Dystrophy

NIH · Sep 2020 – Aug 2026

Closest labs

Michael Lopez

University of Alabama at Birmingham, AL·Genetics·Early-career

Test how over-expressing or knocking down Smad8, a TGF-beta–linked transcription factor elevated in Duchenne muscle, changes muscle cell growth, maturation and regeneration in cell culture and Duchenne mouse models.

Forum Kamdar

University of Minnesota, MN·Biomedical Engineering·Early-career

Build 3D-bioprinted human chambered muscle pumps (hChaMPs) from DMD patient stem cell-derived heart cells and test how progressively increasing pressure alters mechanotransduction and heart function at early and later disease stages.

Renzhi Han

Indiana University-Purdue University-Indianapolis, IN·Pharmacology

Develop an improved triple-vector system in next-generation myotropic adeno-associated virus capsids to reconstitute full-length dystrophin by protein trans-splicing across skeletal, diaphragm, and heart muscle in models of Duchenne muscular dystrophy.

The school

University of Iowa: 32 labs on PI Pages, $703M a year on research, 418 research doctorates a year.

More about University of Iowa

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