FGF21 underlies a hormetic response to metabolic stress in methylmalonic acidemia.

doi:10.1172/jci.insight.124351

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

Community

Back to search

Article

FGF21 underlies a hormetic response to metabolic stress in methylmalonic acidemia.

JCI insight - 6 Dec 2018

Manoli Irini, Sysol Justin R, Epping Madeline W, Li Lina, Wang Cindy, Sloan Jennifer L, Pass Alexandra, Gagné Jack, Ktena Yiouli P, Li Lingli, Trivedi Niraj S, Ouattara Bazoumana, Zerfas Patricia M, Hoffmann Victoria, Abu-Asab Mones, Tsokos Maria G, Kleiner David E, Garone Caterina, Cusmano-Ozog Kristina, Enns Gregory M, Vernon Hilary J, Andersson Hans C, Grunewald Stephanie, Elkahloun Abdel G, Girard Christiane L, Schnermann Jurgen, DiMauro Salvatore, Andres-Mateos Eva, Vandenberghe Luk H, Chandler Randy J, Venditti Charles P

Abstract excerpt

Methylmalonic acidemia (MMA), an organic acidemia characterized by metabolic instability and multiorgan complications, is most frequently caused by mutations in methylmalonyl-CoA mutase (MUT). To define the metabolic adaptations in MMA in acute and chronic settings, we studied a mouse model generated by transgenic expression of Mut in the muscle. Mut-/-;TgINS-MCK-Mut mice accurately replicate the hepatorenal...

Read the complete abstract on PubMed

Topics

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

Share to Fibroblast Growth Factors

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Amino Acid Metabolism, Inborn Errors

Share to Genetic Therapy

Share to Kidney Diseases

Share to Liver Transplantation

Share to Methylmalonyl-CoA Mutase

Share to Stress, Physiological

Share to Transcriptome

Source

Open in PubMed Open full text

Identifiers

PMID: 30518688
DOI: 10.1172/jci.insight.124351
PMCID: PMC6328030

Genes

FGF21

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

FGF21 underlies a hormetic response to metabolic stress in methylmalonic acidemia.PMID 30518688Read ↗

Targeting proximal tubule mitochondrial dysfunction attenuates the renal disease of methylmalonic acidemia.Semantic proximity Read ↗

Integrated multi-omics reveals anaplerotic insufficiency in methylmalonyl-CoA mutase deficiencySemantic proximity Read ↗

Metabolic phenotype of methylmalonic acidemia in mice and humans: the role of skeletal muscle.Semantic proximity Read ↗

Integration of proteomics and metabolomics data in a novel cellular knock out model of methylmalonic acidemiaSemantic proximity Read ↗

Mitochondrial dysfunction in mut methylmalonic acidemia.Semantic proximity Read ↗

Biomarkers to predict disease progression and therapeutic response in isolated methylmalonic acidemia.Semantic proximity Read ↗

In-depth phenotyping reveals common and novel disease symptoms in a hemizygous knock-in mouse model (Mut-ko/ki) of mut-type methylmalonic aciduria.Semantic proximity Read ↗

Untargeted Metabolomics Reveals Distinct Metabolic Profiles in MMA Patients with MUT Gene MutationsSemantic proximity Read ↗

Novel Mouse Models of Methylmalonic Aciduria Recapitulate Phenotypic Traits with a Genetic Dosage Effect.Semantic proximity Read ↗

High-fat diet and FGF21 cooperatively promote aerobic thermogenesis in mtDNA mutator miceSemantic proximity Read ↗

Treatment of metabolic disorders using genomic technologies: Lessons from methylmalonic acidemiaSemantic proximity Read ↗

Impaired mitophagy links mitochondrial disease to epithelial stress in methylmalonyl-CoA mutase deficiencySemantic proximity Read ↗

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