5. Homocystinuria is caused by a defect in cystathionine beta-synthase (or β-synthase), which leads to an accumulation of homocysteine in the blood. This accumulation causes symptoms such as a tall, thin frame, flushed cheeks, and osteoporosis (thinning of the bones). These individuals should limit their intake of proteins that contain methionine, such as egg whites. Using your understanding of biochemistry, explain why people with Homocystinuria should not consume egg whites and other such proteins. ( answer not in a big parahraph small pargaraph very small )
People with Homocystinuria should limit their intake of proteins that contain methionine, such as egg whites, because methionine is a precursor to homocysteine in the body. In individuals with Homocystinuria, there is a defect in cystathionine beta-synthase (or β-synthase), which is an enzyme responsible for converting homocysteine to cystathionine.
As a result of this defect, homocysteine cannot be properly metabolized and accumulates in the blood. This accumulation of homocysteine leads to the symptoms associated with Homocystinuria, including a tall, thin frame, flushed cheeks, and osteoporosis.
Since proteins like egg whites contain methionine, consuming these proteins would further contribute to the accumulation of homocysteine in individuals with Homocystinuria. By limiting their intake of proteins that contain methionine, individuals with Homocystinuria can help reduce the levels of homocysteine in their blood, potentially managing their symptoms and overall health.