Lysosomal and Glycogen Storage Diseases is part of the General Pathology section of Pathology. Practise 10 exam-style MCQs for NEET PG, FMGE, INI-CET and NEET SS, each with the correct answer and a detailed explanation.
About Lysosomal and Glycogen Storage Diseases MCQs
Lysosomal and Glycogen Storage Diseases MCQs centre on Gaucher disease and Niemann-Pick disease, their enzyme defects, hepatosplenomegaly with pancytopenia, and the mannose-6-phosphate tag that routes enzymes to lysosomes. These are high-yield NEET PG questions for FMGE, INI-CET and NEET SS.
Sample Lysosomal and Glycogen Storage Diseases MCQs with answers
Sample MCQ #1
A 10 year old child complaining of abdominal pain and weakness. On examination hepatosplenomegaly noted. In blood investigation pancytopenia was found. Bone marrow shows 'crumbled tissue paper appearance'. This disorder is occur because of accumulation of which of the following molecule.
AGlucocerebroside
BSphingomyelin
CGanglioside
DGalactocerebroside
Show Answer & Explanation ▾
✓ Correct Answer: A. Glucocerebroside
Gaucher’s Disease Most common Lysosomal Storage Disorder Enzyme Defect:- Glucocerebrosidase defect Lysosomes filled with Glucocerebroside No cherry red spot in the macula Hematological features: Pancytopenia, bleeding manifestation Hepatosplenomegaly Bone Pain and Pathological Fractures of long bones X-ray Femur Erlenmeyer Flask Deformity Bone Marrow Biopsy Gaucher cell with Wrinkled paper appearance/crumbled tissue paper appearance. Elevated seruml levls of chitotriosidase, ACE & TRAP, also CCL-18/PARC Reference: Harper's 32nd edition, page 251
Sample MCQ #2
Accumulation of sphingomyelin in phagocytic cells is feature of:
ATay-sachs disease
BGaucher's disease
CNiemann-Pick disease
DDown's syndrome
Show Answer & Explanation ▾
✓ Correct Answer: C. Niemann-Pick disease
Niemann-Pick Disease Autosomal Recessive Biochemical defect Deficient activity of acid sphingomyelinase, a lysosomal enzyme encoded by a gene on chromosome 11 Accumulation of sphingomyelin and other lipids in the monocyte-macrophage system. Clinical features Failure to thrive Hepatosplenomegaly Rapidly progressive neurodegenerative course. Treatment Orthotopic liver transplantation Amniotic cell transplantation Bone marrow transplantation Miglustat A phase I trial of enzyme replacement therapy for type B NPD. Reference: Harper's 32nd edition, page 250-251
Sample MCQ #3
Lysosomal enzymes are tagged with which residue in Golgi apparatus to direct them to lysosome?
AGlucose 6 phosphate
BMannose 6 phosphate
CLecithin
DPhosphatidylserine
Show Answer & Explanation ▾
✓ Correct Answer: B. Mannose 6 phosphate
Mannose 6- phosphate is marker for delivering lysosomal enzymes Lysosomes are membrane-bound organelles containing roughly 40 different acid hydrolases (i.e., enzymes that function best in acidic pH - 5). These hydrolases include proteases, nucleases, lipases, glycosidases, phosphatases, and sulfatases. Lysosomal enzymes are initially synthesized in the ER lumen and then tagged with a mannose-6-phosphate (M6P) residue within the Golgi apparatus. Such M6P-modified proteins are subsequently delivered to lysosomes through trans-Golgi vesicles that express M6P receptors. Reference: Robbins & Cotran Pathologic Basis of Diseases 10 th edition, Chapter - 1 pg 13
Sample MCQ #4
A 48-year-old lady presented with hepatosplenomegaly with pancytopenia. On bone marrow examination, a tissue paper crumpled appearance is seen. Which is the most likely product to have accumulated?
AGlucocerebroside
BSphingomylin
CSulfatide
DGanglioside
Show Answer & Explanation ▾
✓ Correct Answer: A. Glucocerebroside
The clinical feature and bone marrow feature is typical of Gauchers disease. This results due to accumulation of cerebrosideQ inside mononuclear phagocytic cells due to deficiency of ß-glucocerebrosidase. Reference: Robbins & Cotran Pathologic Basics of Diseases 9 th Edition page No.151
What you can practise in Lysosomal and Glycogen Storage Diseases
10 Lysosomal and Glycogen Storage Diseases MCQs on Lysosomal, Glycogen Storage Diseases, General Pathology — each with detailed explanations
Previous-year question practice across NEET PG, FMGE, INI-CET and NEET SS
Progress and performance tracking to measure retention
What do Lysosomal and Glycogen Storage Diseases MCQs cover?
Lysosomal and Glycogen Storage Diseases MCQs centre on Gaucher disease and Niemann-Pick disease, their enzyme defects, hepatosplenomegaly with pancytopenia, and the mannose-6-phosphate tag that routes enzymes to lysosomes. These are high-yield NEET PG questions for FMGE, INI-CET and NEET SS.
How many Lysosomal and Glycogen Storage Diseases questions are there, and which exams do they cover?
MedLadder includes 10 curated MCQs for Lysosomal and Glycogen Storage Diseases under Pathology (General Pathology), covering NEET PG, FMGE, INI-CET and NEET SS preparation.
