Cholic acid as a treatment for cerebrotendinous xanthomatosis in adults

Mandia, Daniele; Chaussenot, Annabelle; Besson, Gérard; Lamari, Foudil; Castelnovo, Giovanni; Curot, Jonathan; Duval, Fanny; Giral, Philippe; Lecerf, Jean‐Michel; Roland, Dominique; Pierdet, Heloise; Douillard, Claire; Nadjar, Yann

doi:10.1007/s00415-019-09377-y

Cited by 36 publications

(35 citation statements)

References 19 publications

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“…Cornuside attenuates apoptosis and ameliorates mitochondrial energy metabolism in rat cortical neurons (Jiang et al, 2009; Jiang et al, 2014). Cholic acid can be used as a treatment for cerebrotendinous xanthomatosis in adults (Mandia et al, 2019). 5-hydroxymethyl-2-furaldehyde (5-HMF) may block scopolamine-induced learning deficit and enhance cognitive function via the activation of NMDA receptor signaling, including CaMKII and ERK, and would be an effective candidate against cognitive disorders, such as Alzheimer’s disease (Lee et al, 2015).…”

Section: Discussionmentioning

confidence: 99%

Bazhu Decoction, a Traditional Chinese Medical Formula, Ameliorates Cognitive Deficits in the 5xFAD Mouse Model of Alzheimer’s Disease

et al. 2019

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Alzheimer’s disease (AD) is the most common neurodegenerative disorder associated with aging. There are currently no effective treatments for AD. Bazhu decoction (BZD), a traditional Chinese medicine (TCM) formula, has been employed clinically to alleviate AD. However, the underlying molecular mechanisms are still unclear. Here we found that middle- and high-doses of BZD ameliorated the behavioral aspects of 5xFAD transgenic mice in elevated plus maze, Y maze and Morris water maze tests. Moreover, BZD reduced the protein levels of BACE1 and PS1, resulting in a reduction of Aβ plaques. We also identified a beneficial effect of BZD on oxidative stress by attenuating MDA levels and SOD activity in the brains of 5xFAD mice. Together, these results indicate that BZD produces a dose-dependent positive effect on 5xFAD transgenic mouse model by decreasing APP processing and Aβ plaques, and by ameliorating oxidative damage. BZD may play a protective role in the cognitive and anxiety impairments and may be a complementary therapeutic option for AD.

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Section: Discussionmentioning

confidence: 99%

Bazhu Decoction, a Traditional Chinese Medical Formula, Ameliorates Cognitive Deficits in the 5xFAD Mouse Model of Alzheimer’s Disease

et al. 2019

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“…One study highlighted the efficacy of using CA, instead of the standard CDCA, with significantly diminished cholestanol levels without elevated transaminases. The adverse effect of altered transaminases and subsequently cholestasis has been shown in the traditional CDCA treatment in a neonatal CTX case [ 118 ]. Another group differentiated indirect bile acid synthesis biomarkers in an extensive long-term study, with significant elevation of plasma cholestanol and serum lathosterol in untreated patients.…”

Section: Bile Acids In Neurodegenerative Diseasesmentioning

confidence: 99%

Bile Acid Signaling in Neurodegenerative and Neurological Disorders

Grant

DeMorrow

2020

IJMS

113

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Bile acids are commonly known as digestive agents for lipids. The mechanisms of bile acids in the gastrointestinal track during normal physiological conditions as well as hepatic and cholestatic diseases have been well studied. Bile acids additionally serve as ligands for signaling molecules such as nuclear receptor Farnesoid X receptor and membrane-bound receptors, Takeda G-protein-coupled bile acid receptor and sphingosine-1-phosphate receptor 2. Recent studies have shown that bile acid signaling may also have a prevalent role in the central nervous system. Some bile acids, such as tauroursodeoxycholic acid and ursodeoxycholic acid, have shown neuroprotective potential in experimental animal models and clinical studies of many neurological conditions. Alterations in bile acid metabolism have been discovered as potential biomarkers for prognosis tools as well as the expression of various bile acid receptors in multiple neurological ailments. This review explores the findings of recent studies highlighting bile acid-mediated therapies and bile acid-mediated signaling and the roles they play in neurodegenerative and neurological diseases.

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“…Other forms of therapies like antisense oligonucleotides, targeted drug therapy, enzyme replacement therapy and stem cell‐based therapy need further evidence before they are proved efficacious in the clinic. Table 6 summarizes the currently recommended therapeutic measures and Table S6 lists the emerging treatment modalities for common and selected disorders 4,62‐95 …”

Section: Treatmentmentioning

confidence: 99%

Genetic disorders with central nervous system white matter abnormalities: An update

et al. 2020

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Several genetic disorders have variable degree of central nervous system white matter abnormalities. We retrieved and reviewed 422 genetic conditions with prominent and consistent involvement of white matter from the literature. We herein describe the current definitions, classification systems, clinical spectrum, neuroimaging findings, genomics, and molecular mechanisms of these conditions. Though diagnosis for most of these disorders relies mainly on genomic tests, specifically exome sequencing, we collate several clinical and neuroimaging findings still relevant in diagnosis of clinically recognizable disorders. We also review the current understanding of pathophysiology and therapeutics of these disorders.

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Cholic acid as a treatment for cerebrotendinous xanthomatosis in adults

Cited by 36 publications

References 19 publications

Bazhu Decoction, a Traditional Chinese Medical Formula, Ameliorates Cognitive Deficits in the 5xFAD Mouse Model of Alzheimer’s Disease

Bazhu Decoction, a Traditional Chinese Medical Formula, Ameliorates Cognitive Deficits in the 5xFAD Mouse Model of Alzheimer’s Disease

Bile Acid Signaling in Neurodegenerative and Neurological Disorders

Genetic disorders with central nervous system white matter abnormalities: An update

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