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Article

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

Molecular cell - 3 Aug 2017

Kang Yilin, Stroud David A, Baker Michael J, De Souza David P, Frazier Ann E, Liem Michael, Tull Dedreia, Mathivanan Suresh, McConville Malcolm J, Thorburn David R, Ryan Michael T, Stojanovski Diana

Abstract excerpt

Acylglycerol kinase (AGK) is a mitochondrial lipid kinase that catalyzes the phosphorylation of monoacylglycerol and diacylglycerol to lysophosphatidic acid and phosphatidic acid, respectively. Mutations in AGK cause Sengers syndrome, which is characterized by congenital cataracts, hypertrophic cardiomyopathy, skeletal myopathy, exercise intolerance, and lactic acidosis. Here we identified AGK as a subunit of the...

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Identifiers

PMID: 28712726
DOI: 10.1016/j.molcel.2017.06.014

Genes

AGK

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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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Sengers Syndrome-Associated Mitochondrial Acylglycerol Kinase Is a Subunit of the Human TIM22 Protein Import Complex.PMID 28712726Read ↗

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

The TIM22 complex regulates mitochondrial one-carbon metabolism by mediating the import of SideroflexinsSemantic proximity Read ↗

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

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