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Title: Jag1  
Author: World Heritage Encyclopedia
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Subject: Tetralogy of Fallot, HES1, CD153, CD109, CD320
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Jagged 1

Rendering based on PDB .
Available structures
PDB Ortholog search: PDBe, RCSB
Symbols  ; AGS; AHD; AWS; CD339; HJ1; JAGL1
External IDs GeneCards:
RNA expression pattern
Species Human Mouse
RefSeq (mRNA)
RefSeq (protein)
Location (UCSC)
PubMed search

JAG1 is a protein associated with Alagille syndrome. JAG1 has also been designated as CD339 (cluster of differentiation 339). The jagged 1 protein encoded by JAG1 is the human homolog of the Drosophilia jagged protein. Human jagged 1 is the ligand for the receptor Notch-1, the latter a human homolog of the Drosophilia jagged receptor notch. Mutations that alter the jagged 1 protein cause Alagille syndrome. Jagged 1 signalling through notch 1 has also been shown to play a role in hematopoiesis.[1]


JAG1 has been shown to interact with NOTCH2.[2][3][4]

See also


  1. ^ "Entrez Gene: JAG1 jagged 1 (Alagille syndrome)". 
  2. ^ Shimizu, K; Chiba S; Saito T; Kumano K; Takahashi T; Hirai H (July 2001). "Manic fringe and lunatic fringe modify different sites of the Notch2 extracellular region, resulting in different signaling modulation". J. Biol. Chem. (United States) 276 (28): 25753–8.  
  3. ^ Shimizu, K; Chiba S; Kumano K; Hosoya N; Takahashi T; Kanda Y; Hamada Y; Yazaki Y; Hirai H (November 1999). "Mouse jagged1 physically interacts with notch2 and other notch receptors. Assessment by quantitative methods". J. Biol. Chem. (UNITED STATES) 274 (46): 32961–9.  
  4. ^ Shimizu, K; Chiba S; Hosoya N; Kumano K; Saito T; Kurokawa M; Kanda Y; Hamada Y; Hirai H (September 2000). "Binding of Delta1, Jagged1, and Jagged2 to Notch2 Rapidly Induces Cleavage, Nuclear Translocation, and Hyperphosphorylation of Notch2". Mol. Cell. Biol. (UNITED STATES) 20 (18): 6913–22.  

Further reading

External links

  • GeneReviews/NCBI/UW/NIH entry on Alagille syndrome
  • OMIM entries on Alagille syndrome

This article incorporates text from the United States National Library of Medicine, which is in the public domain.

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