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MYH2

Domain

The rodlike tail sequence is highly repetitive, showing cycles of a 28-residue repeat pattern composed of 4 heptapeptides, characteristic for alpha-helical coiled coils.

Limited proteolysis of myosin heavy chain produces 1 light meromyosin (LMM) and 1 heavy meromyosin (HMM). HMM can be further cleaved into 2 globular subfragments (S1) and 1 rod-shaped subfragment (S2).

Function

Muscle contraction. Required for cytoskeleton organization (By similarity).

Involvement in disease

Myopathy, proximal, with ophthalmoplegia

MYPOP

A muscular disorder characterized by mild-to-moderate muscle weakness, ophthalmoplegia, and contractures at birth in some patients. Muscle biopsies from patients show predominance of type 1 fibers and small or absent type 2A fibers. The disease is non-progressive or it progresses very slowly. Inheritance is autosomal dominant or recessive.

None

The disease is caused by variants affecting the gene represented in this entry.

Sequence similarities

Belongs to the TRAFAC class myosin-kinesin ATPase superfamily. Myosin family.

Cellular localization

  • Cytoplasm
  • Myofibril
  • Thick filaments of the myofibrils.

Alternative names

  • Myosin-2
  • Myosin heavy chain 2
  • Myosin heavy chain 2a
  • Myosin heavy chain IIa
  • MyHC-2a
  • MyHC-IIa
  • MYHSA2
  • MYH2

Target type

Proteins

Molecular weight

223044Da