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Kids' Health
http://kidshealth.org/parent/medical/genetic/tay_sachs.html#cat140
Healthy babies develop vision, movement, hearing, and other vital functions in part because enzymes clear out fatty protein and other unwanted material that can interfere with growth.
But a baby with Tay-Sachs disease is born without one of those important enzymes, Hexosaminidase A (Hex A). So, as those fatty proteins build up in the brain, they hurt the baby's sight, hearing, movement, and mental development...