Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Altered energy metabolism in Fatal Familial Insomnia cerebral organoids is associated with astrogliosis and neuronal dysfunction.

PLoS genetics - 1 Jan 2023

Foliaki Simote T, Smith Anna, Schwarz Benjamin, Bohrnsen Eric, Bosio Catharine M, Williams Katie, Orrú Christina D, Lachenauer Hailey, Groveman Bradley R, Haigh Cathryn L

Abstract excerpt

Fatal familial insomnia (FFI) is a rare neurodegenerative disease caused by a dominantly inherited single amino acid substitution (D178N) within the prion protein (PrP). No in vitro human brain tissue model for this disease has previously been available. Consequently, how this mutation exerts its damaging effect on brain cells is still unknown. Using CRISPR-Cas9 engineered induced pluripotent stem cells, we made...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Gliosis
Share to Mutation

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Neurodegenerative Diseases

Share to Organoids

Share to Oxidation-Reduction

Share to Prion Diseases

Share to Prion Diseases and Protein Misfolding

Share to Prions

Source

Open in PubMed Open full text

Identifiers

PMID: 36656833
DOI: 10.1371/journal.pgen.1010565
PMCID: PMC9851538

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Altered energy metabolism in Fatal Familial Insomnia cerebral organoids is associated with astrogliosis and neuronal dysfunction.PMID 36656833Read ↗

Progranulin loss induces mitochondrial dysfunction and ferroptosis in human cerebral organoidsSemantic proximity Read ↗

Fatal Familial Insomnia: Clinical Aspects and Molecular Alterations.Semantic proximity Read ↗

APOE4 increases energy metabolism in APOE-isogenic iPSC-derived neuronsSemantic proximity Read ↗

Antioxidants Prevent Iron Accumulation and Lipid Peroxidation, but do not Correct Autophagy Dysfunction or Mitochondrial Bioenergetics in Cellular Models of BPANSemantic proximity Read ↗

APOE genotype-dependent differences in human astrocytic energy metabolismSemantic proximity Read ↗

Development of MAPT S305 mutation models exhibiting elevated 4R tau expression, resulting in altered neuronal and astrocytic functionSemantic proximity Read ↗

C9orf72 Repeat Expansion Induces Metabolic Dysfunction in Human iPSC-Derived Microglia and Modulates Glial–Neuronal CrosstalkSemantic proximity Read ↗

Astrocytic Reactivity Triggered by defective Mitophagy Activates NF-kB Signaling and Causes Neurotoxicity in Frontotemporal Dementia Type 3Semantic proximity Read ↗

PSEN1 Mutant iPSC-Derived Model Reveals Severe Astrocyte Pathology in Alzheimer's DiseaseSemantic proximity Read ↗

IPSC-derived midbrain astrocytes from Parkinson’s disease patients carrying pathogenic <i>SNCA</i> mutations exhibit alpha-synuclein aggregation, mitochondrial fragmentation and excess calcium releaseSemantic proximity Read ↗

Compromised lipid metabolism, mitochondria respiration and neuroprotective effects in iPSC-derived astrocytes from a Smith-Lemli-Opitz syndrome patient.Semantic proximity Read ↗

Astrocyte dysfunction and neuronal network hyperactivity in a CRISPR engineered pluripotent stem cell model of frontotemporal dementiaSemantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.