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Familial variability of cerebrotendinous xanthomatosis lacking typical biochemical findings

Cerebrotendinous xanthomatosis (CTX) is a rare autosomal recessive disorder of bile acid synthesis caused by pathogenic variants in the CYP27A1 gene encoding the mitochondrial enzyme sterol 27‐hydroxylase. Patients with CTX can present with a wide range of symptoms, but most often have evidence of t...

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Pubblicato in:JIMD Rep
Autori principali: Guenzel, Adam J., DeBarber, Andrea, Raymond, Kimiyo, Dhamija, Radhika
Natura: Artigo
Lingua:Inglês
Pubblicazione: John Wiley & Sons, Inc. 2021
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC8100391/
https://ncbi.nlm.nih.gov/pubmed/33977023
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/jmd2.12197
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