C3D1115N Mouse Study: Properdin Role in Atypical Haemolytic Uraemic Syndrome

A research paper describes a mouse model study investigating the role of properdin in atypical haemolytic uraemic syndrome (aHUS). The study involved crossing C3D1115N mice with properdin-deficient mice and treating them with anti-properdin monoclonal antibody therapy, tracking survival and assessing kidney function and pathology. The 1.9 MB PDF document was authored by Grace Mallett and last updated on 2026-04-28.

Use Cases

• Study the effects of properdin deficiency on disease progression in a C3 gain-of-function mouse model based on the described experimental cross.
• Evaluate the therapeutic potential of anti-properdin monoclonal antibody (14E1) treatment for thrombotic microangiopathic anaemia based on the described intervention results.
• Analyze correlations between serum biomarkers, kidney function, and kidney pathology in a mouse model of aHUS based on the described assessment endpoints.
• Investigate C3 and fibrin deposition patterns in kidney tissue using immuno-fluorescence data mentioned in the methods.

Strengths

• The study includes longitudinal survival tracking of mice over a ~6-month period.
• Experimental design involves both genetic (properdin-deficient cross) and therapeutic (anti-properdin antibody) interventions.
• Data includes multiple assessment endpoints: kidney function, serum biomarkers, and kidney pathology.

Limitations

• The dataset is a 1.9 MB PDF; the underlying raw experimental data (e.g., columnar measurements) is not directly accessible.
• Row count and specific data variables are unknown, requiring extraction from the document.
• Description metadata is limited; actual data quality requires manual inspection after download.

Provenance

Source

figshare, authored by Grace Mallett.

Collection Method

Experimental study using a genetically engineered mouse model (C3D1115N) crossed with properdin-deficient mice and treated with monoclonal antibody therapy.

Freshness

Last updated 2026-04-28 04:29:11; freshness should be verified.