Atrial Standstill 1 (ATRST1)

Alias:
Cardiomyopathy, Familial, with Conduction Disturbance
Atrial Cardiomyopathy with Heart Block
Atrst1
Familial Cardiomyopathy with Conduction Disturbance
Primary Idiopathic Myocardial Disease
Atrial Standstill, Digenic
Standstill, Atrial, Type 1
Primary Myocardial Disease
Idiopathic Cardiomyopathy
Idiopathic Cardiopathy
Primary Cardiomyopathy
Cardiomyopathies
Myocardiopathy
Heart Block
Myocardosis
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Basic Information
Medical Symptom
Gene & Mutation
Related Drugs
Disease Model
References Literature
Atrial Standstill 1, also known as cardiomyopathy, familial, with conduction disturbance, is related to atrial standstill and progressive familial heart block, type ia, and has symptoms including angina pectoris, chest pain and edema. An important gene associated with Atrial Standstill 1 is GJA5 (Gap Junction Protein Alpha 5), and among its related pathways/superpathways are DREAM Repression and Dynorphin Expression and Antiarrhythmic Pathway, Pharmacodynamics. The drugs Dolutegravir and Abacavir have been mentioned in the context of this disorder. Affiliated tissues include heart and bone marrow, and related phenotypes are ventricular escape rhythm and paroxysmal atrial fibrillation
Related ID:
MESH:D006327
ICD11:282225286

Basic Information

Inheritance
Age of Onset
Prevalence
Related Gene
Related Mouse Models
Reference
MALACARDS
AD
Unknown
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254
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Medical Symptom

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

Gene & Mutation

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Related Drugs

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No data available

Disease Model

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Name
MGI
Related Gene
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Publications
No data available

References Literature

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PMID
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IF
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