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Article

Acylglycerol Kinase Mutated in Sengers Syndrome Is a Subunit of the TIM22 Protein Translocase in Mitochondria.

Molecular cell - 3 Aug 2017

Vukotic Milena, Nolte Hendrik, König Tim, Saita Shotaro, Ananjew Maria, Krüger Marcus, Tatsuta Takashi, Langer Thomas

Abstract excerpt

Mutations in mitochondrial acylglycerol kinase (AGK) cause Sengers syndrome, which is characterized by cataracts, hypertrophic cardiomyopathy, and skeletal myopathy. AGK generates phosphatidic acid and lysophosphatidic acid, bioactive phospholipids involved in lipid signaling and the regulation of tumor progression. However, the molecular mechanisms of the mitochondrial pathology remain enigmatic. Determining its...

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Source

Identifiers

PMID: 28712724
DOI: 10.1016/j.molcel.2017.06.013

Genes

AGK
TIMM22
TIMM29

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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Acylglycerol Kinase Mutated in Sengers Syndrome Is a Subunit of the TIM22 Protein Translocase in Mitochondria.PMID 28712724Read ↗

Sengers Syndrome-Associated Mitochondrial Acylglycerol Kinase Is a Subunit of the Human TIM22 Protein Import Complex.Semantic proximity Read ↗

Acylglycerol Kinase: Mitochondrial Protein Transport Meets Lipid Biosynthesis.Semantic proximity Read ↗

Characterization of a Novel Splicing Variant in Acylglycerol Kinase (AGK) Associated with Fatal Sengers Syndrome.Semantic proximity Read ↗

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Lack of the mitochondrial protein acylglycerol kinase causes Sengers syndrome.Semantic proximity Read ↗

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Sengers syndrome caused by biallelic TIMM29 variants and RNAi silencing in Drosophila orthologue recapitulates the human phenotype.Semantic proximity Read ↗

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