IPR045579
Acylglycerol kinase, C-terminal
InterPro entry
Short name | AGK_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
Cross References
Contributing Member Database Entry
- Pfam:PF19712