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St. Jude Study Reveals a New Function for an Old Enzyme in Fatal Childhood Disease
Date:7/7/2008

eded products into small building blocks that the cell can reuse or dispose.

The St. Jude team found that in the absence of NEU1, white cell lysosomes are more prone than normal lysosomes to dock and eventually fuse with the cell membrane and subsequently spill their active enzymes into the bone marrow environment. This aberrant behavior hampers the ability of hematopoietic stem cells (HSCs) to be correctly retained within the bone niche. HSCs are immature cells that give rise to all the types of blood cells in the body. The researchers showed that the released enzymes prematurely digest a protein on bone marrow stromal cells called VCAM-1, a molecule that these cells use to hold onto HSCs in the bone marrow.

Deprived of their normal nurturing environment, the HSCs migrate out of the bone marrow and into the spleen, crowding into the organ until it becomes severely enlarged. "Our work represents an unexpected and important clue to one of the prominent clinical manifestations of sialidosis patients," d'Azzo said. "We were surprised to discover that an old, ubiquitous enzyme in lysosomes better known for digesting cellular waste products plays such an important role in a basic biological process that when exacerbated contributes to the outcome of such a terrible disease in children."

In a series of experiments, d'Azzo's team discovered that the way NEU1 regulates the pool of lysosomes destined for lysosomal exocytosis is by cutting off a sugar called sialic acid from a structural protein of the lysosomal membrane, known as LAMP-1. They found that LAMP-1 is involved in the docking of lysosomes at the cell membrane, a prerequisite for these organelles to fuse with the cell membrane and release their content outside the cell. When NEU1 strips the sialic acids off LAMP-1, this protein is rapidly turned over so that its total amount is reduced. Less LAMP-1 at the lysosomal membrane influences the capacity of lysosomes to dock at the cell membrane and t
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SOURCE St. Jude Children's Research Hospital
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