Skip to main content

Kremen1

Function

Receptor for Dickkopf proteins. Cooperates with DKK1/2 to inhibit Wnt/beta-catenin signaling by promoting the endocytosis of Wnt receptors LRP5 and LRP6. In the absence of DKK1, potentiates Wnt-beta-catenin signaling by maintaining LRP5 or LRP6 at the cell membrane. Can trigger apoptosis in a Wnt-independent manner and this apoptotic activity is inhibited upon binding of the ligand DKK1. Plays a role in limb development; attenuates Wnt signaling in the developing limb to allow normal limb patterning and can also negatively regulate bone formation. Modulates cell fate decisions in the developing cochlea with an inhibitory role in hair cell fate specification.

Involvement in disease

Ectodermal dysplasia 13, hair/tooth type

ECTD13

A form of ectodermal dysplasia, a disorder due to abnormal development of two or more ectodermal structures. ECTD13 is an autosomal recessive form characterized by severe oligodontia accompanied by anomalies of hair and skin.

None

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

Cellular localization

  • Cell membrane
  • Single-pass type I membrane protein

Alternative names

  • Kremen protein 1
  • Dickkopf receptor
  • Kringle domain-containing transmembrane protein 1
  • Kringle-containing protein marking the eye and the nose
  • KRM1
  • KREMEN
  • KREMEN1

Target type

Proteins

Molecular weight

51744Da