disease 4,341 words KG: ent-dise-c0a7941f 2026-03-23
kind:diseasesection:diseasesstate:published
Contents

Niemann-Pick Disease Type C (NPC)

Disease Info
Membrane traffickingImpaired endosomal-lysosomal function disrupts receptor signaling and nutrient sensing
Mitochondrial dysfunctionEnergy production deficits and increased reactive oxygen species generation
AutophagyImpaired autophagic flux leading to protein aggregate accumulation and defective mitophagy[@mtorc2019]
Calcium homeostasisDisrupted ER calcium signaling affects neuronal excitability and survival
NeurotransmissionSynaptic vesicle trafficking abnormalities impair synaptic function
DatabasesOMIMOrphanetClinicalTrialsPubMed

Knowledge Graph

Related Hypotheses (23)

Selective Acid Sphingomyelinase Modulation Therapy
Score: 0.65
Membrane Cholesterol Gradient Modulators
Score: 0.52
APOE-Dependent Autophagy Restoration
Score: 0.61
Multi-Modal Stress Response Harmonization
Score: 0.60
AMPK hypersensitivity in astrocytes creates enhanced mitocho
Score: 0.57

Related Analyses (17)

TREM2 agonism vs antagonism in DAM microglia
neurodegeneration · failed
Autophagy-lysosome pathway convergence across neurodegenerat
neurodegeneration · archived
What are the mechanisms by which gut microbiome dysbiosis in
neurodegeneration · completed
What are the mechanisms by which gut microbiome dysbiosis in
neurodegeneration · archived
Protein aggregation cross-seeding across neurodegenerative d
neurodegeneration · archived

Related Experiments (4)

Macroautophagy Dysfunction in PD - Experiment Design
clinical · proposed · Score: 0.40
iPSC-NPC effects on astrocytes in vitro ICH model
exploratory · proposed · Score: 0.90
AMPK knockdown validation experiment
exploratory · proposed · Score: 0.88
iPSC-NPC transplantation in ICH animal model
validation · proposed · Score: 0.85