Article

Loss of function of the mitochondrial peptidase PITRM1 induces proteotoxic stress and Alzheimer’s disease-like pathology in human cerebral organoids

2020-01-27

Abstract excerpt

Mutations in pitrilysin metallopeptidase 1 ( PITRM1 ), a mitochondrial protease involved in mitochondrial precursor processing and degradation, result in a slow-progressive syndrome, characterized by cerebellar ataxia, psychotic episodes and obsessive behavior as well as cognitive decline. To investigate the pathogenetic mechanisms of mitochondrial presequence processing, we employed cortical neurons and cerebral...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: 7ce59ba5-8281-5aa5-a72c-53296bdb16fc
DOI: 10.1101/2020.01.27.919522

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Loss of function of the mitochondrial peptidase PITRM1 induces proteotoxic stress and Alzheimer’s disease-like pathology in human cerebral organoidsDOI 10.1101/2020.01.27.919522Read

Defective PITRM1 mitochondrial peptidase is associated with Aβ amyloidotic neurodegenerationSemantic proximity Read

In-frame deletion in canine PITRM1 is associated with a severe early-onset epilepsy, mitochondrial dysfunction and neurodegenerationSemantic proximity Read

Ataxia Telangiectasia patient-derived neuronal and brain organoid models reveal mitochondrial dysfunction and oxidative stressSemantic proximity Read

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

Alteration of Mitochondrial Integrity as Upstream Event in the Pathophysiology of SOD1-ALSSemantic proximity Read

Defective proteostasis in induced pluripotent stem cell models of frontotemporal lobar degeneration.Semantic proximity Read

Hallmark Molecular and Pathological Features of POLG Disease are Recapitulated in Cerebral Organoids.Semantic proximity Read

<i>SNCA</i> triplication disrupts proteostasis and extracellular architecture prior to neurodegeneration in human midbrain organoidsSemantic proximity Read

Mitochondrial proteolytic stress induced by loss of mortalin function is rescued by Parkin and PINK1.Semantic proximity Read

LONP1 regulation of mitochondrial protein folding provides insight into beta cell failure in type 2 diabetesSemantic proximity Read

Cell growth and mitochondrial anomalies in induced pluripotent stem cells with Presenilin 1 mutationSemantic proximity Read

Amyloid-β peptide induces mitochondrial dysfunction by inhibition of preprotein maturation.Semantic proximity Read