RGD Reference Report - Interferon-gamma-Jak-Stat signaling in pulmonary lymphangioleiomyomatosis and renal angiomyolipoma: a potential therapeutic target. - Rat Genome Database

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Interferon-gamma-Jak-Stat signaling in pulmonary lymphangioleiomyomatosis and renal angiomyolipoma: a potential therapeutic target.

Authors: El-Hashemite, N  Kwiatkowski, DJ 
Citation: El-Hashemite N and Kwiatkowski DJ, Am J Respir Cell Mol Biol. 2005 Sep;33(3):227-30. Epub 2005 Jun 30.
RGD ID: 2298537
Pubmed: PMID:15994429   (View Abstract at PubMed)
PMCID: PMC2715313   (View Article at PubMed Central)
DOI: DOI:10.1165/rcmb.2005-0152RC   (Journal Full-text)

Pulmonary lymphangioleiomyomatosis (LAM) and renal angiomyolipoma (AML) are proliferative lesions that occur in sporadic patients, and at much higher frequency in patients with tuberous sclerosis (TSC). The TSC1 and TSC2 genes play a critical role in their pathogenesis. Here we report a marked decrease in interferon (IFN)-gamma expression in both sporadic and TSC-associated AML and LAM. A marked increase in Stat1 expression and phosphorylation at Ser 727, and in phospho-Tyr705-Stat3 levels, was also seen in both AML and LAM tissues. Our results demonstrate that the IFN-gamma-Jak-Stat pathway is perturbed in TSC-related and sporadic LAM and AML, and suggest that IFN-gamma has potential therapeutic benefit for treatment of those lesions.



RGD Manual Disease Annotations    Click to see Annotation Detail View

  
Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
STAT1Humanangiomyolipoma  IEP protein:increased serine phosphorylation:kidneyRGD 
STAT3Humanangiomyolipoma  IEP protein:increased tyrosine phosphorylation:kidneyRGD 
Stat1Ratangiomyolipoma  ISOSTAT1 (Homo sapiens)protein:increased serine phosphorylation:kidneyRGD 
Stat1Mouseangiomyolipoma  ISOSTAT1 (Homo sapiens)protein:increased serine phosphorylation:kidneyRGD 
Stat3Ratangiomyolipoma  ISOSTAT3 (Homo sapiens)protein:increased tyrosine phosphorylation:kidneyRGD 
Stat3Mouseangiomyolipoma  ISOSTAT3 (Homo sapiens)protein:increased tyrosine phosphorylation:kidneyRGD 
STAT1Humanlymphangioleiomyomatosis  IEP protein:increased serine phosphorylation:lungRGD 
STAT3Humanlymphangioleiomyomatosis  IEP protein:increased tyrosine phosphorylation:lungRGD 
Stat1Mouselymphangioleiomyomatosis  ISOSTAT1 (Homo sapiens)protein:increased serine phosphorylation:lungRGD 
Stat1Ratlymphangioleiomyomatosis  ISOSTAT1 (Homo sapiens)protein:increased serine phosphorylation:lungRGD 
Stat3Ratlymphangioleiomyomatosis  ISOSTAT3 (Homo sapiens)protein:increased tyrosine phosphorylation:lungRGD 
Stat3Mouselymphangioleiomyomatosis  ISOSTAT3 (Homo sapiens)protein:increased tyrosine phosphorylation:lungRGD 

Objects Annotated

Genes (Rattus norvegicus)
Stat1  (signal transducer and activator of transcription 1)
Stat3  (signal transducer and activator of transcription 3)

Genes (Mus musculus)
Stat1  (signal transducer and activator of transcription 1)
Stat3  (signal transducer and activator of transcription 3)

Genes (Homo sapiens)
STAT1  (signal transducer and activator of transcription 1)
STAT3  (signal transducer and activator of transcription 3)


Additional Information