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 mitochondrial interactome, we have identified AGK as a constituent of the TIM22 complex in the mitochondrial inner membrane. AGK assembles with TIMM22 and TIMM29 and supports the import of a subset of multi-spanning membrane proteins. The function of AGK as a subunit of the TIM22 complex does not depend on its kinase activity. However, enzymatically active AGK is required to maintain mitochondrial cristae morphogenesis and the apoptotic resistance of cells. The dual function of AGK as lipid kinase and constituent of the TIM22 complex reveals that disturbances in both phospholipid metabolism and mitochondrial protein biogenesis contribute to the pathogenesis of Sengers syndrome.
Pubmed ID: 28712724 RIS Download
Publication data is provided by the National Library of Medicine ® and PubMed ®. Data is retrieved from PubMed ® on a weekly schedule. For terms and conditions see the National Library of Medicine Terms and Conditions.
This polyclonal targets Smac/DIABLO
View all literature mentionsThis polyclonal targets CHCHD3
View all literature mentionsThis monoclonal targets Cytochrome c
View all literature mentionsThis polyclonal targets LMNA
View all literature mentionsThis unknown targets Rabbit IgG (H&L) Antibody CY5.5 Conjugated Pre-Adsorbed
View all literature mentionsThis polyclonal targets Mitofilin
View all literature mentionsThis monoclonal targets OxPhos Complex IV subunit II (cytochrome oxidase subunit II)
View all literature mentionsThis polyclonal targets VDAC1
View all literature mentionsThis monoclonal targets Adenine Nucleotide Translocase 1 antibody [5F51BB5AG7]
View all literature mentionsThis monoclonal targets Human MFN1
View all literature mentionsThis polyclonal targets SDHC antibody
View all literature mentionsThis polyclonal targets TIMM17B
View all literature mentionsThis polyclonal targets Acylglycerol Kinase antibody
View all literature mentionsThis polyclonal targets CANX
View all literature mentionsThis monoclonal targets Cytochrome c
View all literature mentionsThis monoclonal targets OPA1
View all literature mentionsThis monoclonal targets ATP Synthase beta
View all literature mentionsThis monoclonal targets ATP5A
View all literature mentionsThis polyclonal targets YME1L1
View all literature mentionsThis monoclonal targets anti Heat shock protein 70 / HSP70
View all literature mentionsThis monoclonal targets alpha-Tubulin antibody produced in mouse
View all literature mentionsThis monoclonal targets PDI
View all literature mentionsThis polyclonal targets TIM22
View all literature mentionsThis monoclonal targets FLAG
View all literature mentionsSoftware for automatic general image analysis. It provides fully automatic analysis of 1-D gels including lane creation, background subtraction, band detection, molecular weight calibration, quantity calibration, and normalization. Editing tools are provided for cropping, rotating, and filtering images.
View all literature mentionsOpen source Java based image processing software program designed for scientific multidimensional images. ImageJ has been transformed to ImageJ2 application to improve data engine to be sufficient to analyze modern datasets.
View all literature mentionsCell line HeLa is a Cancer cell line with a species of origin Homo sapiens
View all literature mentionsStatistical analysis software that combines scientific graphing, comprehensive curve fitting (nonlinear regression), understandable statistics, and data organization. Designed for biological research applications in pharmacology, physiology, and other biological fields for data analysis, hypothesis testing, and modeling.
View all literature mentionsSoftware environment and programming language for statistical computing and graphics. R is integrated suite of software facilities for data manipulation, calculation and graphical display. Can be extended via packages. Some packages are supplied with the R distribution and more are available through CRAN family.It compiles and runs on wide variety of UNIX platforms, Windows and MacOS.
View all literature mentionsA quantitative proteomics software package for analyzing large-scale mass-spectrometric data sets. It is a set of algorithms that include peak detection and scoring of peptides, mass calibration, database searches for protein identification, protein quantification, and provides summary statistics.
View all literature mentionsSoftware for automatic general image analysis. It provides fully automatic analysis of 1-D gels including lane creation, background subtraction, band detection, molecular weight calibration, quantity calibration, and normalization. Editing tools are provided for cropping, rotating, and filtering images.
View all literature mentionsOpen source Java based image processing software program designed for scientific multidimensional images. ImageJ has been transformed to ImageJ2 application to improve data engine to be sufficient to analyze modern datasets.
View all literature mentionsSoftware for automatic general image analysis. It provides fully automatic analysis of 1-D gels including lane creation, background subtraction, band detection, molecular weight calibration, quantity calibration, and normalization. Editing tools are provided for cropping, rotating, and filtering images.
View all literature mentions