[Expression of Notch signaling pathway in children with Henoch-Schönlein purpura and its correlation with inflam-matory cytokines].

OBJECTIVES

To investigate the expression of Notch signaling pathway-related genes in peripheral blood mononuclear cells (PBMCs) of children with Henoch-Schönlein purpura (HSP) and their correlation with inflammatory cytokine levels.

METHODS

A total of 66 children with initial-onset HSP (30 in the complex HSP group and 36 in the simple HSP group) treated at the Guangzhou Women and Children's Medical Center, along with 28 healthy controls, were enrolled. The messenger RNA (mRNA) expression levels of NOTCH1-NOTCH4, JAG1, JAG2, and HES1 in PBMCs were detected by quantitative reverse transcription polymerase chain reaction (qRT-PCR). Serum levels of IL-1β, IL-4, IL-6, IL-17, interferon-γ, and tumor necrosis factor (TNF)-α were measured by enzyme-linked immunosorbent assay (ELISA). Spearman correlation analysis was performed to evaluate the relationships between NOTCH1/NOTCH2 mRNA levels and the mRNA levels of the ligand-coding gene JAG1, the downstream target gene HES1, as well as inflammatory cytokine levels.

RESULTS

Compared with the healthy control group, both the simple and complex HSP groups showed significantly elevated mRNA levels of NOTCH1, NOTCH2, JAG1, and HES1, as well as increased serum levels of IL-17, interferon-γ, and TNF-α (all P<0.05). Furthermore, the mRNA levels of NOTCH1, NOTCH2, and HES1 were significantly higher in the complex HSP group than those in the simple HSP group (all P<0.05). NOTCH1 mRNA levels were positively correlated with JAG1 and HES1 mRNA levels, as well as with interferon-γ and TNF-α levels (all P<0.05). NOTCH2 mRNA levels were positively correlated with IL-17 and interferon-γ levels (both P<0.05).

CONCLUSIONS

The Notch signaling pathway is significantly activated during the acute phase of HSP and may participate in the immunopathological process by regulating the secretion of pro-inflammatory cytokines. The mRNA expression levels of NOTCH1 and NOTCH2 may correlate with disease severity, potentially serving as reference indicators for disease assessment.