Niemann-Pick disease type C

14 papers2 findings

Animals14

Effects

1

Cellular senescence

upin mice1

1 study

Niemann-Pick disease type C increases cellular senescence in mice.
“microglia with increased cell numbers exhibit a cellular senescent phenotype in an NPC mouse model.”
Animalsin an NPC mouse modelbrain
Molecular and histological characterizations reveal two distinct senescent microglia populations in Niemann-Pick disease type C mouse model
GeroScience10 Apr 2026

More on Cellular senescence

Upstream

1

NR1H2

downin mice1

1 study

NR1H2 protects against Niemann-Pick disease type C in mice.
“deletion of the nuclear receptor, liver X receptor beta (LXRbeta), had an adverse effect on progression of the disease.”
Animalsin NPC1 mice
Liver X receptor activation enhances cholesterol loss from the brain, decreases neuroinflammation, and increases survival of the NPC1 mouse
The Journal of neuroscience : the official journal of the Society for Neuroscience26 Dec 2007

Latest

3 in 12 months

Brain Communications13 Sep

Developmental brain programmes and biomarker activation in Alzheimer's disease

ReviewMechanisms

bioRxiv29 Jul

NPC1 deficiency engages a lysosome-genome-immune program linked to neurodegeneration and cellular aging signatures

Preprint: experiments in miceNeurodegenerative disease

GeroScience11 Apr

Molecular and histological characterizations reveal two distinct senescent microglia populations in Niemann-Pick disease type C mouse model

Experiments in miceCellular senescence

GSA 2024 Abstract1 Jan

MODULATING CELLULAR SENESCENCE AND MTOR PATHWAY: THE THERAPEUTIC POTENTIAL OF FINGOLIMOD IN NPC DISEASE

Experiments in miceInterventions

PNAS4 May

Acat1/Soat1 knockout extends the mutant Npc1 mouse lifespan and ameliorates functional deficiencies in multiple organelles of mutant cells

Experiments in miceMechanisms

European Journal of Pharmacology24 Jan

Beneficial effects of primidone in Niemann-Pick disease type C (NPC)-model cells and mice: Reduction of unesterified cholesterol levels in cells and extension of lifespan in mice

Experiments in miceInterventions

Nature Biomedical Engineering14 Jan

Cytosine and adenine base editing of the brain, liver, retina, heart and skeletal muscle of mice via adeno-associated viruses

Experiments in miceEye

EMBO Reports18 Sep

NPC1 enables cholesterol mobilization during long-term potentiation that can be restored in Niemann-Pick disease type C by CYP46A1 activation

Experiments in miceInterventions

Human Molecular Genetics1 Sep

AAV9 intracerebroventricular gene therapy improves lifespan, locomotor function and pathology in a mouse model of Niemann-Pick type C1 disease

Experiments in miceInterventions

Journal of Lipid Research4 Jan

AAV9-NPC1 significantly ameliorates Purkinje cell death and behavioral abnormalities in mouse NPC disease

Experiments in miceInterventions