D
IPR045579

Acylglycerol kinase, C-terminal

InterPro entry
Short nameAGK_C
Overlapping
homologous
superfamilies
 

Description

This domain corresponds to the C-terminal of mitochondrial acylglycerol kinase (AGK, also known as MuLK)
[1, 2, 3]
. AGK is a metazoan-specific protein integrated into the mitochondrial inner membrane through a short N-terminal transmembrane domain. This large C-terminal domain is adjacent the kinase domain and it is oriented to the intermembrane space. AGK is a subunit of the human TIM22 complex which stabilises the complex and regulates the import and assembly of mitochondrial carrier proteins, a function independent of its kinase activity
[1, 2]
. Disturbances in both functions of AGK (phospholipid metabolism and mitochondrial protein biogenesis) contribute to the pathogenesis of Sengers syndrome, which is characterised by cataracts, hypertrophic cardiomyopathy, and skeletal myopathy
[1, 2]
.

References

1.Sengers Syndrome-Associated Mitochondrial Acylglycerol Kinase Is a Subunit of the Human TIM22 Protein Import Complex. Kang Y, Stroud DA, Baker MJ, De Souza DP, Frazier AE, Liem M, Tull D, Mathivanan S, McConville MJ, Thorburn DR, Ryan MT, Stojanovski D. Mol Cell 67, 457-470.e5, (2017). PMID: 28712726

2.Acylglycerol Kinase Mutated in Sengers Syndrome Is a Subunit of the TIM22 Protein Translocase in Mitochondria. Vukotic M, Nolte H, Konig T, Saita S, Ananjew M, Kruger M, Tatsuta T, Langer T. Mol Cell 67, 471-483.e7, (2017). PMID: 28712724

3.MuLK, a eukaryotic multi-substrate lipid kinase. Waggoner DW, Johnson LB, Mann PC, Morris V, Guastella J, Bajjalieh SM. J Biol Chem 279, 38228-35, (2004). PMID: 15252046

Cross References

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