Hexosaminidase Total Unit Converter
Convert between nmol/min/mL, U/L
Also known as: Total Hexosaminidase, β-N-Acetylglucosaminidase, Hex Total, Total Hex
Convert Hexosaminidase Total
What is Hexosaminidase Total?
Total hexosaminidase measures the combined activity of two lysosomal enzymes, hexosaminidase A (Hex A) and hexosaminidase B (Hex B), in your blood. These enzymes break down complex fats called GM2 gangliosides and other glycosphingolipids inside cells.
Hex A is made of an alpha and a beta subunit (encoded by the HEXA and HEXB genes), while Hex B is made of two beta subunits. Both enzymes work inside lysosomes, the cell's recycling compartments, to prevent harmful buildup of fatty substances.
This test is most commonly used to screen for and diagnose Tay-Sachs disease and Sandhoff disease, and to identify carriers of these conditions. It is also reported alongside the hexosaminidase A percentage to help distinguish between the two disorders.
Where Does Hexosaminidase Total Come From?
Hexosaminidase enzymes are produced inside lysosomes of cells throughout the body, with particularly high activity in the liver, brain, and white blood cells (leukocytes).
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