Chapter 12, Problem 12.8PS

(a)

Summary Introduction

To determine: Whether the given statement, “Impaired metabolism of glycolipids that causes mental deterioration” is common to most lysosomal storage diseases (M), true of a specific lysosomal storage disease (S), or not true of any lysosomal storage diseases (N).

Introduction: Accumulation of metabolites such as polysaccharides or lipids in the lysosomes causes lysosomal diseases. Deficiency of certain proteins, as well as enzymes, causes the accumulation of these metabolites. Defects in proteins essential for transportation of degradation products leads to accumulation of degraded products in the lysosomes. Most lysosomal storage diseases are fatal.

(b)

Summary Introduction

To determine: Whether the given statement, “Leads to accumulation of degradation products in the lysosome” is common to most lysosomal storage diseases (M), true of a specific lysosomal storage disease (S), or not true of any lysosomal storage diseases (N).

Introduction: Accumulation of metabolites such as polysaccharides or lipids in the lysosomes causes lysosomal diseases. Deficiency of certain proteins, as well as enzymes, causes the accumulation of these metabolites. Defects in proteins essential for transportation of degradation products leads to accumulation of degraded products in the lysosomes. Most lysosomal storage diseases are fatal.

(c)

Summary Introduction

To determine: Whether the given statement, “Leads to accumulation of excessive amounts of glycogen in the lysosome” is common to most lysosomal storage diseases (M), true of a specific lysosomal storage disease (S), or not true of any lysosomal storage diseases (N).

Introduction: Accumulation of metabolites such as polysaccharides or lipids in the lysosomes causes lysosomal diseases. Deficiency of certain proteins, as well as enzymes, causes the accumulation of these metabolites. Defects in proteins essential for transportation of degradation products leads to accumulation of degraded products in the lysosomes. Most lysosomal storage diseases are fatal.

(d)

Summary Introduction

To determine: Whether the given statement, “Results from an inability to regulate the synthesis of glycosaminoglycans” is common to most lysosomal storage diseases (M), true of a specific lysosomal storage disease (S), or not true of any lysosomal storage diseases (N).

Introduction: Accumulation of metabolites such as polysaccharides or lipids in the lysosomes causes lysosomal diseases. Deficiency of certain proteins, as well as enzymes, causes the accumulation of these metabolites. Defects in proteins essential for transportation of degradation products leads to accumulation of degraded products in the lysosomes. Most lysosomal storage diseases are fatal.

(e)

Summary Introduction

To determine: Whether the given statement, “Results from an absence of functional acid hydrolases” is common to most lysosomal storage diseases (M), true of a specific lysosomal storage disease (S), or not true of any lysosomal storage diseases (N).

Introduction: Accumulation of metabolites such as polysaccharides or lipids in the lysosomes causes lysosomal diseases. Deficiency of certain proteins, as well as enzymes, causes the accumulation of these metabolites. Defects in proteins essential for transportation of degradation products leads to accumulation of degraded products in the lysosomes. Most lysosomal storage diseases are fatal.

(f)

Summary Introduction

To determine: Whether the given statement, “Results in accumulation of lysosomes in the cell” is common to most lysosomal storage diseases (M), true of a specific lysosomal storage disease (S), or not true of any lysosomal storage diseases (N).

Introduction: Accumulation of metabolites such as polysaccharides or lipids in the lysosomes causes lysosomal diseases. Deficiency of certain proteins, as well as enzymes, causes the accumulation of these metabolites. Defects in proteins essential for transportation of degradation products leads to accumulation of degraded products in the lysosomes. Most lysosomal storage diseases are fatal.

(g)

Summary Introduction

To determine: Whether the given statement, “Symptoms include muscle weakness and mental retardation” is common to most lysosomal storage diseases (M), true of a specific lysosomal storage disease (S), or not true of any lysosomal storage diseases (N).

Introduction: Accumulation of metabolites such as polysaccharides or lipids in the lysosomes causes lysosomal diseases. Deficiency of certain proteins, as well as enzymes, causes the accumulation of these metabolites. Defects in proteins essential for transportation of degradation products leads to accumulation of degraded products in the lysosomes. Most lysosomal storage diseases are fatal.

(h)

Summary Introduction

To determine: Whether the given statement, “Triggers proliferation of organelles containing catalase” is common to most lysosomal storage diseases (M), true of a specific lysosomal storage disease (S), or not true of any lysosomal storage diseases (N).

Introduction: Accumulation of metabolites such as polysaccharides or lipids in the lysosomes causes lysosomal diseases. Deficiency of certain proteins, as well as enzymes, causes the accumulation of these metabolites. Defects in proteins essential for transportation of degradation products leads to accumulation of degraded products in the lysosomes. Most lysosomal storage diseases are fatal.