Deficiency of lysosomal maltase causes?
**Core Concept**
The question is testing the understanding of lysosomal enzyme deficiency, specifically maltase, which is a key enzyme involved in carbohydrate metabolism. Maltase breaks down maltose into two glucose molecules within the lysosomes of cells.
**Why the Correct Answer is Right**
Deficiency of lysosomal maltase leads to Pompe disease, also known as glycogen storage disease type II. This condition is characterized by the accumulation of glycogen within lysosomes due to the inability to break it down. Glycogen is a complex carbohydrate that is stored in the liver and muscles. The deficiency of maltase results in the accumulation of glycogen, leading to cellular damage and subsequent organ dysfunction.
**Why Each Wrong Option is Incorrect**
**Option A:** This option is incorrect because deficiency of lysosomal maltase is not directly related to Pompe disease, which is the correct answer. Deficiency of other lysosomal enzymes can lead to different glycogen storage diseases, such as glycogen storage disease type III.
**Option B:** This option is incorrect because deficiency of sucrase-isomaltase is related to a different condition, known as sucrase-isomaltase deficiency, which affects the small intestine and leads to malabsorption of carbohydrates.
**Option C:** This option is incorrect because deficiency of lactase is related to lactose intolerance, which is a condition that affects the ability to digest lactose in dairy products.
**Option D:** This option is incorrect because deficiency of amylase is related to pancreatic insufficiency, which can lead to malabsorption of carbohydrates.
**Clinical Pearl / High-Yield Fact**
Pompe disease is an autosomal recessive disorder, meaning that both parents must be carriers of the mutated gene to pass it on to their offspring.
**Correct Answer:** C. Pompe disease