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PAX7

Function

Transcription factor that is involved in the regulation of muscle stem cells proliferation, playing a role in myogenesis and muscle regeneration.

Involvement in disease

Rhabdomyosarcoma 2

RMS2

A form of rhabdomyosarcoma, a highly malignant tumor of striated muscle derived from primitive mesenchymal cells and exhibiting differentiation along rhabdomyoblastic lines. Rhabdomyosarcoma is one of the most frequently occurring soft tissue sarcomas and the most common in children. It occurs in four forms: alveolar, pleomorphic, embryonal and botryoidal rhabdomyosarcomas.

None

The gene represented in this entry is involved in disease pathogenesis. A chromosomal aberration involving PAX7 is found in rhabdomyosarcoma. Translocation t(1;13)(p36;q14) with FOXO1. The resulting protein is a transcriptional activator.

Congenital myopathy 19

CMYO19

An autosomal recessive muscular disorder characterized by infantile onset of progressive muscular atrophy, hypotonia, ptosis, scoliosis and dysmorphic facial features. Disease severity is variable, ranging from mild to severe.

None

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

Post-translational modifications

Acetylation at Lys-105 and Lys-139 by KAT8 is required for high-level transcription factor activity. Deacetylated by SIRT2.

Sequence similarities

Belongs to the paired homeobox family.

Cellular localization

  • Nucleus

Alternative names

  • HUP1
  • HUP1
  • PAX7
  • Paired box protein Pax-7
  • HuP1

Target type

Proteins

Primary research area

Epigenetics

Molecular weight

55119Da

We found 9 products in 2 categories

Primary Antibodies

Target

Application

Reactive species

Proteins & Peptides

Target

Species of origin

Nature