disease 3,136 words KG: ent-dise-e177291e 2026-03-29
kind:diseasesection:diseasesstate:published
Contents

Sialidosis

Disease Info
PrevalenceApproximately 1 in 1,000,000 to 1 in 4,000,000 live births worldwide 6
Type I (cherry-red spot myoclonus)More common than type II, typically presenting in the second or third decade of life
Type II (infantile/juvenile)Rarer, with earlier onset and more severe phenotype
Gender distributionEqual male and female prevalence (autosomal recessive inheritance)
Geographic distributionWorldwide, with higher reported prevalence in populations with higher rates of consanguinity
Ethnic clusteringSome populations show founder effects leading to local clustering of cases 7
Missense mutationsThe most common type, often resulting in reduced enzyme stability or activity
Nonsense mutationsCreating premature stop codons and truncated proteins
Splice-site mutationsLeading to exon skipping or intron retention
Small deletions/insertionsCausing frameshifts and premature termination 9
DatabasesOMIMOrphanetClinicalTrialsPubMed

Knowledge Graph

Related Hypotheses (1)

Astrocytic Lactate Shuttle Enhancement for Grid Cell Bioener
Score: 0.41

Related Analyses (3)

Autophagy-lysosome pathway convergence across neurodegenerat
neurodegeneration · archived
Protein aggregation cross-seeding across neurodegenerative d
neurodegeneration · archived
Metabolic reprogramming in neurodegenerative disease
neurodegeneration · completed

Related Experiments (13)

Animal Model Comparison for Neurodegenerative Disease Therap
clinical · proposed · Score: 0.40
Endocannabinoid System Dysfunction Validation in Parkinson's
clinical · proposed · Score: 0.40
Glymphatic-Circadian Axis Enhancement Therapy for Parkinson'
clinical · proposed · Score: 0.40
Levodopa-Induced Dyskinesias Mechanism — Experiment Design
validation · proposed · Score: 0.40
Gut Microbiome-Derived Metabolites in Alpha-Synuclein Propag
clinical · proposed · Score: 0.40