DNAJB6-related limb-girdle muscular dystrophy D1

What is DNAJB6-related limb-girdle muscular dystrophy D1?

A subtype of autosomal dominant limb-girdle muscular dystrophy characterized by an adult-onset of slowly progressive, proximal pelvic girdle weakness, with none, or only minimal, shoulder girdle involvement, and absence of cardiac and respiratory symptoms. Mild to moderate elevated creatine kinase serum levels and gait abnormalities are frequently observed.

Other condition names

  • Autosomal dominant limb-girdle muscular dystrophy type 1D
  • DNAJB6-related LGMD D1
  • LGMD type 1D
  • LGMD1D
  • Limb-girdle muscular dystrophy type 1D

Inheritance type

Autosomal dominant


  • Worldwide: <1 in 1,000 000

Age of Onset

  • Adult
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on "https://www.orphadata.com". Data version 1.3.16 / 4.1.7.