Treatment with 2, 4-dihydroxybenzoic acid prevents FSGS progression and renal fibrosis in podocyte-specific Coq6 knockout mice
E Widmeier, M Airik, H Hugo, D Schapiro… - Journal of the …, 2019 - journals.lww.com
Background Although studies have identified> 55 genes as causing steroid-resistant
nephrotic syndrome (SRNS) and localized its pathogenesis to glomerular podocytes, the
disease mechanisms of SRNS remain largely enigmatic. We recently reported that
individuals with mutations in COQ6, a coenzyme Q (also called CoQ 10, CoQ, or
ubiquinone) biosynthesis pathway enzyme, develop SRNS with sensorineural deafness,
and demonstrated the beneficial effect of CoQ for maintenace of kidney function. Methods To …
nephrotic syndrome (SRNS) and localized its pathogenesis to glomerular podocytes, the
disease mechanisms of SRNS remain largely enigmatic. We recently reported that
individuals with mutations in COQ6, a coenzyme Q (also called CoQ 10, CoQ, or
ubiquinone) biosynthesis pathway enzyme, develop SRNS with sensorineural deafness,
and demonstrated the beneficial effect of CoQ for maintenace of kidney function. Methods To …
