HYDROXYCHLOROQUINE AMELIORATES THE SEVERITY IN AN X-LINKED ALPORT SYNDROME MOUSE MODEL

Certificate Output Instructions

For best output, select "Paper Size" as "A4" and "Margin" as "0" or "None".

To save or print to PDF, please select Print Destination > Save as PDF, enable Background Graphics under "More Settings", then click "Save".

Presented the abstract " "
(Abstract co-author(s): )

Back

E-Poster Presentation

During the congress, E-Posters will be accessible to all participants on the congress website 24/7, as well as in the E-poster stations in the congress center.

Preparing your E-Poster

Please review the E-Poster format requirements carefully when preparing your E-Poster. Should your E-Poster not meet the mentioned requirements, it may not be displayed as described above.

E-Poster Submission Deadline

Please prepare and upload your E-Poster no later than March 14, 2026 11.59PM CET. After this date, you will no longer be able to prepare and upload your E-poster and it will not be displayed and accessible on the congress website.

E-Poster Format Requirements

PDF file
Layout: Portrait (vertical orientation)
One page only (Dim A4: 210 x 297mm or PPT)
E-Poster can be prepared in PowerPoint (one (1) PowerPoint slide) but must be saved and submitted as PDF file.
File Size: Maximum file size is 2 Megabytes (2 MB)
No hyperlinks, animated images, animations, and slide transitions
Language: English
Include your abstract number
E-posters can include QR codes, tables and photos

E-Poster

Abstract Title *

HYDROXYCHLOROQUINE AMELIORATES THE SEVERITY IN AN X-LINKED ALPORT SYNDROME MOUSE MODEL

Please follow the instructions below to input your abstract title.

Abstract titles should be brief and reflect the content of the abstract.

The title will not be accepted if it exceeds 25 words.
Type in CAPITAL LETTERS.
Lowercase may be used for abbreviations only, for example, mRNA.

Co-author 1

LEI SUN sly0527@126.com Shanghai Children’s Hospital Department of Nephrology and Rheumatology Shanghai China *

Co-author 2

Xin-Yu Kuang kuangxy@shchildren.com.cn Shanghai Children’s Hospital Department of Nephrology and Rheumatology Shanghai China -

Co-author 3

Xiu-Li Gong gongxl@shchildren.com.cn Shanghai Children’s Hospital Shanghai Institute of Medical Genetics Shanghai China -

Co-author 4

Qin Cai caiq@shchildren.com.cn Shanghai Children’s Hospital Shanghai Institute of Medical Genetics Shanghai China -

Co-author 5

Fanyi Zeng fzeng@vip.163.com Shanghai Children’s Hospital Shanghai Institute of Medical Genetics Shanghai China -

Co-author 6

Wen-Yan Huang huangwenyan@sjtu.edu.cn Shanghai Children’s Hospital Department of Nephrology and Rheumatology Shanghai China -

Co-author 7

Co-author 8

Co-author 9

Co-author 10

Co-author 11

Co-author 12

Co-author 13

Co-author 14

Co-author 15

Introduction

Alport syndrome is the most common inherited glomerular disease. At present, no cure has been made available for this devastating disorder. This study aims to assess the effectiveness and potential mechanism of hydroxychloroquine (HCQ) in treating XLAS mice.

Methods

The XLAS mouse model was genotyped as COL4A5 p.G5X. During treatment, the mice were monitored for urine protein/creatinine levels, liver and kidney function. Renal histopathological examination scores were performed at the end of treatment. The HCQ treatment survival analysis group (Col4a5-/- male mice) was established to investigate the lifespan. The study employed transcriptome analysis of renal tissues to investigate the potential mechanisms underlying the effects of HCQ on the XLAS mouse.

Results

For COL4A5-/+ female mice, 8 weeks of treatment with high-dose HCQ, low-dose HCQ, or benazepril significantly reduced urinary protein excretion. The high-dose HCQ treatment group exhibited significantly lower urinary protein excretion compared to both the low-dose HCQ and benazepril treatment groups. In COL4A5-/- male mice, urinary protein excretion was significantly decreased after high-dose HCQ treatment and benazepril treatment. In addition, HCQ significantly decreased the blood creatinine level, reduced glomerulosclerosis and fibrosis, and prolonged the survival period of the XLAS mouse model.

Transcriptome analysis found that 58 genes related to cytokine-cytokine receptor interaction were significantly altered after HCQ treatment of CLO4A5-/- male mice, of which 48 (82.8%) were upregulated and 10 (17.2%) downregulated. These results indicate that the CLO4A5-/-mice showed significant activation of cytokine-cytokine receptor interaction signaling pathways in renal tissues. After HCQ was administered, IL22RA1 in CLO4A5-/-male mice shifted from a significantly upregulated to a significantly downregulated state.

Conclusion

HCQ significantly reduced proteinuria in the XLAS mouse model. HCQ could alleviate renal injury, and extend the survival time of CLO4A5-/-male mice. HCQ may play a role in treating XLAS by regulating IL22RA1 and its cytokine-cytokine receptor signaling pathways.

Kewords