CD339 Jagged 1 (Alagille syndrome), JAG1, JAGL1, HJ1
Molecule TypeAntigen ExpressionMolecular Weight
Min / Max
Non-lineage Restricted Molecule
Type 1 glycoprotein
Stromal Cell
Epithelia
Bone Marrow
Heart
Placenta
Carcinoma Cell
Kidney
Epithelial Cell
150 / 150

Expression
CD339 is expressed on stromal bone marrow and epithelial cells.  There is wide expression in adult and fetal tissues.  Expression is also in heart, placenta and kidney with weaker expression in lung, skelatal muscle and pancreas.  Expression is found in metastatic and nonmetastatic squamous epithelia and is upregulated in squamous cell carcinoma and in situ and invasive adenocarcinoma.

Structure
MOLECULAR FAMILY NAME: Belong to the Drosophilia jagged ligands family.

CD339 is a single-pass type-1 1185 aa glycoprotein.  It contains a 22 aa signal peptide, an 1034 aa extracellular domain which contains 1 DSL (Delta, Serrate and LagZ) domain, 15 EGF-like domains, 8 of which bind calcium and a juxtamembrane that is cysteine-rich, 9 potential N-linked glycosylation sites, a 26 aa span the transmembrane domain and an 125 aa intracellular cytoplasmic domain.  The EGF-like domains 4-7, 10, 11, 14 and 15 bind calcium.  Human jagged 1 is the ligand for the receptor Notch 1. 

MOLECULAR MASS

POST-TRANSCRIPTIONAL MODIFICATION: No information.

POST-TRANSLATIONAL MODIFICATION

CD399 has 9 potential N-linked glycoprotein sites.

Ligands
LIGANDS AND MOLECULE ASSOCIATED WITH CD339

CD339 binds the Notch receptor (Notch1, Notch2 and Notch 3).

Function
CD339 acts as a ligand for some Notch receptors and is involved in Notch signaling.  CD339 binding to the Notch receptor triggers a cascade of proteolytic cleavage that eventually leads to the release of the intracellular part of the receptor from the membrane, allowing it to translocate to the nucleus and activate transcription factors that plays key roles in cell differentiation, morphogenesis, proliferation and in the regulation of cell-fate decisions during hematopoiesis.  It seems to be involved in cell fate decisions during hematopoiesis and during early and late stages of mammalian cardiovascular system. CD339 mediated signaling inhibits myoblast differentiation, and enhances fibroblast growth factor-induced angiogensis (in vitro). 

BIOCHEMICAL ACTIVITY: No information.

DISEASE RELEVANCE AND FUNCTION OF CD339 IN INTACT ANIMAL

Mutations that alter the jagged 1 protein causes Alagille syndrome, an autosomal dominant developmental disorder and a major form of chronic liver disease in children that affects the structure of the heart, skeleton, eye, kidney and other organs.  Defects in CD339 also causes tetralogy of  Fallot, a congenital heart anomaly characterized by pulmonary stenosis, ventricular septal defects, dextroposition of the aorta and hypertrophy of the right ventricle. 

Comments
MOLECULAR INTERACTIONS-
PROTEINS AND DNA ELEMENTS WHICH REGULATE TRANSCRIPTION OF CD339: No information.

SUBSTRATES: No information.

ENZYMES WHICH MODIFY CD339: No information.

ADDITIONAL INSIGHTS

For further information see Varnum-Finney, B. et al (1998) Blood 91: 4084-4091.


Database accession numbers
AnimalPIRSWISSPROTEMGBL/GENBANK
 
HumanEntrezgene 182P78504
Antibodies

Revised June 25, 2008


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