Cerebrotendinous Xanthomatosis (CTX)

Alias:
Ctx
Cholestanol Storage Disease
Xanthomatosis, Cerebrotendinous
Cerebral Cholesterinosis
Sterol 27-Hydroxylase Deficiency
Xanthomatosis Cerebrotendinous
Van Bogaert-Scherer-Epstein Disease
Cerebrotendinous Cholesterinosis
Cholestanolosis
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Basic Information
Medical Symptom
Gene & Mutation
Related Drugs
Disease Model
References Literature
Cerebrotendinous Xanthomatosis, also known as ctx, is related to lipid storage disease and sitosterolemia, and has symptoms including angina pectoris, cerebellar ataxia and muscle spasticity. An important gene associated with Cerebrotendinous Xanthomatosis is CYP27A1 (Cytochrome P450 Family 27 Subfamily A Member 1), and among its related pathways/superpathways are Metabolism and Metapathway biotransformation Phase I and II. The drugs Chenodeoxycholic acid and Benzocaine have been mentioned in the context of this disorder. Affiliated tissues include skin and brain, and related phenotypes are visual impairment and juvenile cataract
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Basic Information

Inheritance
Age of Onset
Prevalence
Related Gene
Related Mouse Models
Reference
MALACARDS
AR
Newborn
<1/1000000
22
175
180

Medical Symptom

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Categorization
Description
HPO Frequency
Orphanet Frequency
HPO Source Accession
No data available

Gene & Mutation

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Function
Score
Mutations
No data available

Related Drugs

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CAS Number
Status
Phase
No data available

Disease Model

Category
Name
MGI
Related Gene
Strain of Origin
Publications
No data available

References Literature

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PMID
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IF
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