Background of Relationship between Lysosomal Lipidome Changes and Lysosomal storage disease
Lipid metabolism plays a key role in biology. They form lipid bilayers and determine the structure and function of various membranes in eukaryotic cells. Abnormal lysosomal lipid catabolism caused by mutations in enzyme and transporter genes is the cause of many inherited diseases, often referred to as lysosomal storage disorders. Lipids accumulate in the lysosomes of these patients, leading to an imbalance in the cellular lipid profile and impairing lysosomal activity. Lipids play a crucial role as a biomarker and therapeutic target in our body's metabolism and in the lipid remodeling observed in pathophysiological conditions. Therefore monitoring the levels of individual lipid classes or lipid species can be used to assess disease progression. Lysosomal lipidome analysis allows for higher resolution identification of cell surface lipid changes or abnormal accumulation in cargo lysosomal storage disorders and neurodegenerative diseases.
Fig. 1. Cartoon illustrating a model of plasma lipid dyshomeostasis in lysosomal storage disorders. (Fuller M, et al., 2018)
Our Lysosomal Lipidome Change Analysis Services in Lysosomal Storage Disease
We not only provide lysosomal lipidomics services to understand lipid metabolism, but also for the discovery of novel lipid metabolites associated with normal and disease states in humans. Here, CD BioSciences focuses on the analysis of lysosomal lipidome changes in a variety of neurodegenerative lysosomal storage disorders, such as neuronal ceroid lipofuscinoses (NCL), Niemann-Pick type C (NPC), Alzheimer's disease (AD) and frontotemporal lobar degeneration (FTLD-TDP) with progranulin (PGRN) deficiency.
Our engineers have successfully established knockout mouse models of these diseases to analyze changes in the lysosomal lipidome. In addition, we developed multiple strategies to analyze lysosomal lipid changes, allowing quantitative and qualitative changes in lysosomal lipid molecular composition to be identified spatially with higher resolution and sensitivity.
- Analysis of the lipidome of knockout cells using birdshot lipidomics.
- Identify changes in lysosomal lipid molecule species after perturbation by fingerprinting.
- Dynamic labeling of lipids by pulse-tracking experiments to provide real-time dynamic images of lipid fluxes in lysosomes.
Why Choose Us
- Enables automated, quantitative, high-throughput assays.
- Enables more complex metabolic flux dynamics studies.
- Comprehensively determine lipid metabolism at the molecular level in disease.
- Advanced technologies, including gas chromatography (GC), HPLC, TLC, MS, NMR, spectroscopic ESI-MS and MALDI-MS.
- Data completeness and availability.
CD BioSciences can meet any reasonable needs of our clients, taking time and budget into consideration for you. Our aim is to be customer-centric and to provide the highest quality services to customers. Our customer service representatives are enthusiastic and trustworthy 24 hours a day, 7 days a week. If you are interested in our services, please feel free to contact us for more information or a detailed discussion.
References
- Fuller M, Futerman A H. (2018) The brain lipidome in neurodegenerative lysosomal storage disorders[J]. Biochemical and biophysical research communications. 504(3): 623-628.
- Tharkeshwar A K, Trekker J, Vermeire W, et al. (2017) A novel approach to analyze lysosomal dysfunctions through subcellular proteomics and lipidomics: the case of NPC1 deficiency[J]. Scientific reports. 7(1): 1-20.