Gerstmann-Straussler Disease (GSD)

Alias:
Gerstmann-Straussler-Scheinker Syndrome
Gerstmann-Straussler-Scheinker Disease
Cerebral Amyloid Angiopathy, Prnp-Related
Prion Dementia
Cerebellar Ataxia, Progressive Dementia, and Amyloid Deposits in Cns
Subacute Spongiform Encephalopathy, Gerstmann-Straussler Type
Amyloidosis, Cerebral, with Spongiform Encephalopathy
Gsd
Gss
Encephalopathy, Subacute Spongiform, Gerstmann-Straussler Type
Subacute Spongiform Encephalopathy Gerstmann-Straussler Type
Cerebral Amyloidosis with Spongiform Encephalopathy
Gluthathione Synthetase Deficiency
Gerstmann Straussler Syndrome
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Basic Information
Medical Symptom
Gene & Mutation
Related Drugs
Disease Model
References Literature
Gerstmann-Straussler Disease, also known as gerstmann-straussler-scheinker syndrome, is related to progressive myoclonus epilepsy and prion disease, and has symptoms including cerebellar ataxia, muscle rigidity and muscle spasticity. An important gene associated with Gerstmann-Straussler Disease is PRNP (Prion Protein (Kanno Blood Group)), and among its related pathways/superpathways are Alzheimer's disease and miRNA effects and Presenilin-Mediated Signaling. Affiliated tissues include brain and cerebellum, and related phenotypes are cognitive impairment and gait ataxia
Related ID:
MESH:D016098
ICD11:406818835

Basic Information

Inheritance
Age of Onset
Prevalence
Related Gene
Related Mouse Models
Reference
MALACARDS
AD
Adult
<1/1000000
16
349
66

Medical Symptom

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Description
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HPO Source Accession
No data available

Gene & Mutation

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Related Drugs

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Disease Model

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MGI
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References Literature

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