Increased HA/CD44/TGFβ signaling implicates in renal fibrosis of a Col4a5 mutant Alport mice.

ABSTRACT: X-linked Alport syndrome (XLAS) caused by X-linked COL4A5 gene mutation is a hereditary disease that affects mainly the kidney. XLAS patients, especially males whose single copy of the COL4A5 gene is disrupted, suffer from a life-threatening renal disease, the mechanism of which remains unclear. Renal fibrosis is a characteristic pathology observed in XLAS kidney tissue. However, the molecular path from COL4A5 loss-of-function to fibrotic pathology is largely unknown. On the basis of a previously established XLAS mouse model, our study revealed an activated CD44-TGFβ signaling known to strongly promote fibrosis, along with an increased level of low molecular weight hyaluronan (LMW-HA) instead of high molecular weight hyaluronan (HMW-HA), to activate CD44-dependent TGFβ signaling in XLAS re

SUBMITTER: Bao Y 

PROVIDER: S-EPMC11905560 | biostudies-literature | 2025 Mar

REPOSITORIES: biostudies-literature