Answer 1 of 1 Done 1. Lysosomal storage diseases or mitochondrial disorders are
ID: 141328 • Letter: A
Question
Answer 1 of 1 Done 1. Lysosomal storage diseases or mitochondrial disorders are a kind of metabolic disorders. Mutations leading to lack of functional protein/complex hinder the basic metabolic functions associated with the organelle. Metabolic burden leads to the cell death and often leads to organ failure in mitochondrial diseases 2. Mitochondria is solely inherited from mother. 3. OMIM top hits for Leigh syndrome #1 MTATP6 gene #2 MTND5 gene #3 MTND3 gene #4 MT-TK gene #5 MTCO3 gene All the listed gene are often associated witlh Mitochondrial disorders. Moreover all the genes listed here are encoded by mtDNA hence can be corrected through mitochondrial transplantation. 4. ATP synthase (Complex V) of mitochondria comprises of almost 10-16 subunit of protein partly encoded by nuclear DNA and partly by mtDNA. Hence it id definitely hetero-oligomeric NOTE: First 4 subparts of the entire uestionaire have been answered. Feel free to comment on the answers provided. Thank vouExplanation / Answer
Lysosomal storage diseases are inherited metabolic disorders caused by defects in lysosomal functions. Lysosomes are organelles which contain specific enzymes that can digest a variety of biomolecules. Lysosomal disorders are caused by defects in enzymes required for the metabolism of lipid, glycoproteins etc. So, the lysosomal storage diseases interfere with the normal metabolism of the cell.
Mitochondrial disordes are caused by defects in mitochondria, organelle which generates ATP for the cell. The mitochondrial disorders can be due to the mutation in mitochondrial DNA or nuclear DNA encoding mitochondrial genes. These disorders affect the normal cellular metabolism. These disorders can affect almost any organ and the severity of the disease also vary.
2. It's true that mitochondria are solely inherited from mother, so if mitochondrial disorders are caused by mutations in mitochondrial DNA it will show maternal inheritance. However, if it is caused by mutations in nuclear DNA it can get inherited in autosomal dominant, autosomal recessive or X-linked.
3. there are more than 75 genes associated with Leigh Syndrome. mostly people with this disorder have the mutation in nuclear DNA but approx 20% of the cases have mutations in mitochondrial DNA. The genes listed are encoded by mitochondrial DNA so in that case, Mitochondrial transplantation could help.
4. As ATP synthase is partly encoded by nuclear DNA and partly by mt DNA, we can call it hetero-oligomeric.