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CLUAP1 (IFT38)

CLUAP1 (IFT38)

Clusterin-associated protein 1 (also known as IFT38); required for ciliogenesis and cilia maintenance. Associates with the IFT complex B and undergoes intraflagellar transport. Knockout in zebrafish causes photoreceptor cell death by 5 days post-fertilization.

Henüz Hedefli Tedavi Yok

Kromozomal Lokus

16p13.3

Kalıtım

Otozomal Resesif

Sıklık

<1% LCA vakalarının

Temel Klinik Özellikler

  • 1No official LCA subtype number assigned — candidate LCA gene
  • 2IFT-B complex component required for ciliogenesis
  • 3Hypomorphic mutations identified in LCA patients
  • 4Zebrafish knockout confirms photoreceptor degeneration