Hexosaminidase A is an enzyme found in the body that plays a crucial role in breaking down certain fatty substances called gangliosides. It is primarily active in the brain and other tissues, helping to maintain healthy cellular function by preventing the accumulation of these complex lipids. Deficiency of Hexosaminidase A leads to a genetic disorder known as Tay-Sachs disease, which results in severe neurological problems. This condition is inherited in an autosomal recessive manner, meaning that both parents must carry the gene mutation for their child to be affected.