GM1-gangliosidosis
Definition
GM1-gangliosidosis is a lysosomal storage condition caused by a reduction or the absence in the amount of the enzyme, beta-galactosidase, in cells. This condition has been referred to by other names such as Norman-Landing disease, Gangliosidosis-GM1 beta-galactosidase-1 deficiency, Hurler-variant, pseudo-Hurler disease, Tay-Sachs disease with visceral involvement, and GLB1 deficiency.
Description
Lysosomes are structures found inside cells that contain specific proteins and enzymes that help digest or breakdown many of the complex biological substances found within the cells. After the lysosomes digest these substances, the remnants are then released from the cell. The role of the lysosome is to keep the inside of the cell clean and to help the cell function normally.
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