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A study by researchers at Baylor College of Medicine, the Jan and Dan Duncan Neurological Research Institute at Texas Children’s Hospital and collaborating institutions is the first to associate neuronal activity to the levels of sphingolipids, a type of fat, in brain cells. Furthermore, disruption of sphingolipid metabolism led to significant neuronal damage and neurodegeneration in animal models, revealing a new molecular perspective to the cause of Gaucher’s disease. Published in Science Advances, the work offers novel opportunities to develop therapies for this and other neurodegenerative disorders.

“Neurodegenerative disorders affect millions worldwide. Research on these conditions has largely focused on perturbations in protein metabolism. However, practice medicine there is emerging evidence that disruptions in lipid metabolism — particularly those that perturb the synthesis or breakdown of sphingolipids — also contribute significantly to the progressive neuronal toxicity observed in neurodegeneration,” said corresponding author Dr. Hugo J. Bellen, distinguished service professor of molecular and human genetics at Baylor and investigator and chair in neurogenetics at the Jan and Dan Duncan Neurological Research Institute at Texas Children’s.

“This is a path-breaking study that has uncovered many previously unknown aspects of how sphingolipids are regulated,” Bellen said. “It has revealed a close relationship between glycosphingolipid metabolism and rare as well as common neurological conditions. Moreover, it offers valuable insights into the molecular cause of Gaucher disease that has opened possible avenues to develop potential therapies for this and several other neurodegenerative disorders, including Parkinson’s disease.”

Accumulation of glucosylceramide leads to Gaucher disease

Proteins and lipids are broken down by enzymes in intracellular structures or organelles called lysosomes, which serve as cellular trash compactors or recycling centers. Any perturbations in this cleanup process allows metabolic waste products to accumulate in the lysosomes, which can cause a progressive degeneration of neurons, brain cells that are particularly vulnerable to lysosomal dysfunction.

Gaucher disease is a rare metabolic and lysosomal storage disorder that affects approximately 6,000 individuals in the United States. These individuals have a mutation in the Glucosylceramides Beta (GBA1) gene that encodes the enzyme glucocerebrosidase that catalyzes the conversion of a sphingolipid called glucosylceramide (GlcCer) to ceramide. In the absence of this enzyme, GlcCer accumulates in various organs including the brain, liver, bone marrow and spleen.

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