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A rare renal disease characterized by glomerular nephropathy with hematuria progressing to end-stage renal disease (ESRD), frequently associated with sensorineural deafness, and occasionally with ocular anomalies.
Empagliflozin reduces glomerular hyperfiltration while Sairei-to (Stephania tetrandra, Atractylodes, etc.) components tetrandrine and atractylodin modulate complement activation (C3, C5a) and reduce IL-1β-mediated inflammatory damage to remaining functional nephrons
Goji berry polysaccharides upregulate LAMP2A and HSC70 expression to enhance chaperone-mediated autophagy, facilitating clearance of misfolded collagen IV α-chains while promoting residual functional collagen network preservation through stress-responsive autophagy pathways
Astragaloside IV and cycloastragenol synergize with ACE inhibitors to preserve podocyte integrity by activating telomerase (TERT) and enhancing nephrin (NPHS1) expression while reducing TGF-β1-mediated glomerular basement membrane thickening
Withanoside IV and withanoside VI selectively inhibit MMP-9 activity, preventing degradation of already-compromised collagen IV α-chains in Alport syndrome, while withanolide A promotes autophagy-mediated clearance of misfolded collagen aggregates via mTOR pathway modulation
Plant-derived nanovesicles from turmeric loaded with synthetic miR-200 family mimics target TGF-β1/Smad3 pathway to reduce fibrosis, while curcumin enhances exosomal uptake and provides additional anti-inflammatory effects via NF-κB inhibition in podocytes and mesangial cells
Eurycomanone and other quassinoids from Tongkat Ali may stabilize defective COL4A3/COL4A4/COL4A5 collagen networks by promoting proper protein folding through heat shock protein 47 (HSP47) upregulation and reducing endoplasmic reticulum stress via IRE1α pathway modulation