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ACE-031

Muscle Growth

Also known as: ActRIIB-Fc, Activin Receptor Type IIB Fusion Protein

Half-life: ~10-15 days (Fc-fusion)

Last reviewed:  ·  Published:

Muscle Growth

Overview

ACE-031 is a recombinant fusion protein composed of the extracellular domain of the human activin type IIB receptor (ActRIIB) linked to the Fc portion of an IgG antibody. It works as a "ligand trap": by binding myostatin (GDF-8), activin A, GDF-11, and other TGF-β superfamily ligands in circulation, it prevents them from activating the ActRIIB receptor on muscle cells. The result is a net release of myostatin's tonic inhibition of muscle growth, producing rapid increases in muscle mass and strength in preclinical models.

ACE-031 was developed by Acceleron Pharma in collaboration with Shire as a clinical candidate for Duchenne muscular dystrophy (DMD). Early-phase trials in healthy volunteers showed significant increases in lean mass and thigh muscle volume after just one to four doses. However, the Phase 2 trial in DMD boys was halted in 2011 after several participants developed minor capillary leak (gum bleeding, mild nosebleeds) — likely an off-target consequence of trapping the ActRIIB ligands that also regulate vasculature. The program was discontinued.

ACE-031 remains one of the most thoroughly studied myostatin pathway inhibitors and is widely sold by research-chemical vendors. It is biologically distinct from synthetic short-peptide myostatin inhibitors (which usually fail to produce comparable effects) because it is a full recombinant biologic with high binding affinity. Researchers should be aware that the capillary leak signal observed in the DMD trial is a real safety concern, not an idiosyncrasy.

History

ACE-031 was developed in the late 2000s by Acceleron Pharma, a Cambridge MA biotech focused on TGF-β superfamily therapeutics. The Phase 1 trial in healthy postmenopausal women, published in Muscle & Nerve in 2013, demonstrated dose-dependent increases in lean mass after single doses. The Phase 2 trial in pediatric DMD patients began in 2010 but was suspended in 2011 following the capillary leak observations. Acceleron later pivoted its myostatin work into related programs (notably luspatercept/Reblozyl, an ActRIIB ligand trap for anemia, which was eventually approved). ACE-031 itself was never advanced past Phase 2.

Effects

  • Inhibits myostatin and related TGF-β superfamily ligands
  • Rapid increases in lean muscle mass in clinical trials
  • Increased thigh muscle volume by MRI
  • Increased serum markers of muscle growth
  • Reduced fat mass in some trial subjects

Side Effects

  • Capillary leak (gum bleeds, nosebleeds, telangiectasias) — clinical trial finding
  • Injection-site reactions
  • Headache
  • Potential off-target effects on vasculature and bone
  • Limited long-term safety data

Tolerability

Short-term tolerability in the Phase 1 healthy-volunteer trial was acceptable, with the principal adverse events being injection-site reactions and headache. The decision to halt the Phase 2 DMD program after observing mild bleeding suggests the safety margin is narrower than for peptide-based GH secretagogues, and that ActRIIB ligand trapping has off-target consequences for vascular integrity that may scale with cumulative dose. Researchers using ACE-031 should monitor for unusual bleeding and avoid it in subjects with vascular fragility.

Dosing Ranges

Research / muscle volume increase

Dose Range

0.5-3 mg/kg

Frequency

IV or SubQ every 2-4 weeks

Duration

Per protocol

Dosing information is for educational purposes only. Consult a healthcare professional before using any peptide.

Reconstitution

Preparation Details

Typical Vial Size

1 mg

Water Type

Bacteriostatic water (BAC water)

Mixing Volume

1 mL

Half-Life

~10-15 days (Fc-fusion)

Molecular Weight

~90 kDa

Reconstitute gently — avoid shaking, which can denature the Fc-fusion protein. Store at 2-8°C and use within 7-14 days for best activity.

Calculate ACE-031 dose

Where to buy ACE-031

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Regulatory Status

FDA Status

Not FDA approved. Clinical development halted in 2011 after capillary leak observations.

Legal Status

Unregulated research chemical. Prohibited by WADA.

USA

Not approved

Clinical development discontinued by Acceleron

EU

Not approved

Not authorized as medicinal product

UK

Not approved

Classified as research chemical

Australia

Not approved

TGA has not authorized

Canada

Not approved

Not authorized for human use

Cited Studies

A soluble activin type IIB receptor improves function in a mouse model of amyotrophic lateral sclerosis

Morrison BM, Lachey JL, Warsing LC, Ting BL, Pullen AE, Underwood KW, Kumar R, Sako D, Grinberg A, Wong V, Colantuoni E, Seehra JS, Wagner KR

Experimental Neurology (2009)

Preclinical study demonstrating that soluble ActRIIB-Fc (the prototype of ACE-031) improved muscle function and survival in an SOD1-ALS mouse model, supporting development of myostatin-pathway inhibitors for neuromuscular disease.

View Study →

A randomized, double-blind, placebo-controlled, single ascending dose study of ACE-031 in healthy postmenopausal women

Attie KM, Borgstein NG, Yang Y, Condon CH, Wilson DM, Pearsall AE, Kumar R, Willins DA, Seehra JS, Sherman ML

Muscle & Nerve (2013)

First-in-human Phase 1 study showing single doses of ACE-031 produced dose-dependent increases in lean mass and thigh muscle volume with acceptable short-term tolerability.

View Study →

Inhibition of myostatin in adult mice increases skeletal muscle mass and strength

Whittemore LA, Song K, Li X, et al.

Biochemical and Biophysical Research Communications (2003)

Foundational study establishing that pharmacological inhibition of myostatin in adult animals produces rapid muscle hypertrophy, providing the rationale for the entire ActRIIB ligand-trap drug class including ACE-031.

View Study →

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