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9 Results Found

  • Review
  • Open Access
82 Citations
9,260 Views
19 Pages

The Emerging and Diverse Roles of Bis(monoacylglycero) Phosphate Lipids in Cellular Physiology and Disease

  • Megan R. Showalter,
  • Anastasia L. Berg,
  • Alexander Nagourney,
  • Hailey Heil,
  • Kermit L. Carraway and
  • Oliver Fiehn

29 October 2020

Although understudied relative to many phospholipids, accumulating evidence suggests that bis(monoacylglycero)phosphate (BMP) is an important class of regulatory lipid that plays key roles in lysosomal integrity and function. BMPs are rare in most ma...

  • Review
  • Open Access
35 Citations
7,282 Views
14 Pages

Lipids as Emerging Biomarkers in Neurodegenerative Diseases

  • Justin Wei,
  • Li Chin Wong and
  • Sebastian Boland

21 December 2023

Biomarkers are molecules that can be used to observe changes in an individual’s biochemical or medical status and provide information to aid diagnosis or treatment decisions. Dysregulation in lipid metabolism in the brain is a major risk factor...

  • Article
  • Open Access
5 Citations
3,457 Views
16 Pages

Decrease in Myelin-Associated Lipids Precedes Neuronal Loss and Glial Activation in the CNS of the Sandhoff Mouse as Determined by Metabolomics

  • Emmanuelle Lecommandeur,
  • Maria Begoña Cachón-González,
  • Susannah Boddie,
  • Ben D. McNally,
  • Andrew W. Nicholls,
  • Timothy M. Cox and
  • Julian L. Griffin

30 December 2020

Sandhoff disease (SD) is a lysosomal disease caused by mutations in the gene coding for the β subunit of β-hexosaminidase, leading to deficiency in the enzymes β-hexosaminidase (HEX) A and B. SD is characterised by an accumulation of g...

  • Review
  • Open Access
43 Citations
8,438 Views
27 Pages

20 August 2021

Here, we present the main features of human acid sphingomyelinase (ASM), its biosynthesis, processing and intracellular trafficking, its structure, its broad substrate specificity, and the proposed mode of action at the surface of the phospholipid su...

  • Article
  • Open Access
60 Citations
9,450 Views
17 Pages

21 October 2019

Illuminating the comprehensive lipid profiles after dietary supplementation of polyunsaturated fatty acids (PUFAs) is crucial to revealing the tissue distribution of PUFAs in living organisms, as well as to providing novel insights into lipid metabol...

  • Article
  • Open Access
8 Citations
5,064 Views
27 Pages

Increased Alveolar Heparan Sulphate and Reduced Pulmonary Surfactant Amount and Function in the Mucopolysaccharidosis IIIA Mouse

  • Tamara L. Paget,
  • Emma J. Parkinson-Lawrence,
  • Paul J. Trim,
  • Chiara Autilio,
  • Madhuriben H. Panchal,
  • Grielof Koster,
  • Mercedes Echaide,
  • Marten F. Snel,
  • Anthony D. Postle and
  • Sandra Orgeig
  • + 2 authors

8 April 2021

Mucopolysaccharidosis IIIA (MPS IIIA) is a lysosomal storage disease with significant neurological and skeletal pathologies. Respiratory dysfunction is a secondary pathology contributing to mortality in MPS IIIA patients. Pulmonary surfactant is cruc...

  • Article
  • Open Access
494 Views
14 Pages

ATP13A2-Mediated Spermine Export Modulates Lipid Catabolism in the Endolysosomal System of SH-SY5Y Cells

  • Alejandra L. Marcos,
  • Mariela M. Gironacci and
  • Felicitas de Tezanos Pinto

Dysfunction of the membrane transporter P5B-ATPase 13A2 (ATP13A2) has been linked to neurodegenerative disorders, while its overexpression has been associated with colorectal cancer. ATP13A2 localizes to lysosomes and late endosomes, where it exports...

  • Article
  • Open Access
43 Citations
5,551 Views
23 Pages

26 August 2020

Parkinson’s disease (PD) is characterized by the loss of dopaminergic neurons from the substantia nigra (SN) that project to the dorsal striatum (caudate-putamen). To better understand the molecular mechanisms underlying PD, we performed combin...

  • Article
  • Open Access
21 Citations
8,007 Views
18 Pages

Polyphenol-rich foods exhibit anti-allergic/-inflammatory properties. As major effector cells of allergies, mast cells undergo degranulation after activation and then initiate inflammatory responses. Key immune phenomena could be regulated by the pro...