What is the end product of the reactions involved in lysosomal storage disorders?

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The end product of the reactions involved in lysosomal storage disorders is the accumulation of undigested substrates.

Lysosomal storage disorders arise from genetic defects that lead to deficiencies in lysosomal enzymes. These enzymes are responsible for breaking down various macromolecules, such as glycoproteins, glycolipids, and polysaccharides. When these enzymes are not functioning correctly or are absent, the substrates that are normally degraded accumulate inside the lysosomes. This accumulation can cause cellular dysfunction, tissue damage, and a variety of symptoms depending on the specific disorder and the substances that are not being processed.

The other options do not accurately represent the primary consequence of lysosomal storage disorders. A deficit of metabolic energy refers to energy loss rather than the specific buildup of undigested materials. Cellular proliferation can be influenced by a variety of factors and is not a direct result of lysosomal enzyme deficiencies. Lastly, a reduction in lysosomal size contradicts the nature of these disorders, as the lysosomes often become enlarged due to the accumulation of substrates. Thus, the correct understanding emphasizes that the hallmark of lysosomal storage disorders is indeed the accumulation of undigested substrates.

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