Chenodeoxycholic acid inhibits increased cholesterol and cholestanol synthesis in patients with cerebrotendinous Xanthomatosis☆
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Rare monogenic disorders of cholesterol metabolism
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2021, Life SciencesCitation Excerpt :Thus, it is also used clinically for the treatment of hypertriglyceridemia and hyperlipoproteinemia [36,37]. Treatment with CDCA can effectively supple the deficiency of CDCA, and improve clinical symptoms of CTX including cholesterol/cholestanol accumulation and bile acids reduction [38–40]. Notably, although early studies indicated that DCA and CDCA inhibit pro-inflammatory cytokines, interleukin 1β (IL-1β), IL-6 and tumor necrosis factor alpha (TNF-α) production by bacterial lipopolysaccharide (LPS)-stimulated macrophages [41], but the treatment of CDCA on rheumatoid arthritis (RA) via a single intravenous infusions dose of 1–2 g was both temporary and inadequate [42].
Cerebrotendinous xanthomatosis, sitosterolemia, Smith-Lemli-Opitz syndrome and the seminal contributions of Gerald Salen, MD (1935–2020)
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2021, Molecular Genetics and Metabolism ReportsCitation Excerpt :Findings that are more specific are bilateral hyperintensities of the dentate nuclei, cerebral and cerebellar white matter [6]. Treatment with CDCA can improve symptoms of CTX by direct inhibition of CYP7A1 and negative feedback on cholesterol biosynthesis, thereby reducing accumulation of toxic metabolites [7,8]. Combination of CDCA with inhibitors of HMG-CoA reductase further reduce cholestanol levels and improves clinical signs [9].
Metabolic profiling in serum, cerebrospinal fluid, and brain of patients with cerebrotendinous xanthomatosis
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A preliminary report of this investigation was presented at the annual meeting of the American Federation for Clinical Research, April 29, 1972. Clin. Res., 20::465.