A gene mutation in a cell results in a non-functional MSF protein, explain the role of the MSF protein and the consequences of this loss of function for a mitochondrial matrix protein synthesized in the cytosol.
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A gene mutation in a cell results in a non-functional MSF protein, explain the role of the MSF protein and the consequences of this loss of function for a mitochondrial matrix protein synthesized in the cytosol.
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- Leigh syndrome is characterized by psychomotor regression: that is, the progressive loss of mental andmovement abilities. Patients also suffer from lacticacidosis, a condition in which mitochondrial respiration is deficient, so their tissues metabolize glucoseanaerobically, leading to the buildup of lactate. Somepatients with Leigh syndrome have a mutation in themitochondrial gene MT-CO3, which encodes a subunit of the electron transport complex cytochromec oxidase. Other patients diagnosed with Leigh syndrome have a loss-of-function mutation in the nucleargene SURF1, which encodes a factor needed for theassembly of this same enzyme complex.a. How can the same symptoms result from mutationsin a mitochondrial gene and from mutations in anuclear gene?In a rare inherited disorder, called Wilson’s disease, excessive amounts of copper accumulate in liver and brain tissue. A prominent symptom of the disease is the deposition of copper in greenishbrown layers surrounding the cornea, called Kayser–Fleischer rings. A defective ATP-dependent protein that transports copper across cell membranes causes Wilson’s disease. Apparently, the copper transport protein is required to incorporate copper into ceruloplasmin and to excrete excess copper. In addition to a diet low in copper, Wilson’s disease is treated with zinc sulfate and the chelating agent penicillamine (p. 148). Describe how these treatments work. [Hint: Metallothionein has a greater affinity for copper than for zinc.]The DNA located inside of mitochondria exhibits approximately ten times the mutation rate seen in nuclear DNA. Provide an explanation as to why this is the case and what are the effects of this higher mutation rate of mitochondrial DNA on disease processes?
- Glucose-6-phosphatase is located inside the endoplasmic reticulum. Describe the probable symptoms of a defect in G6P transport across the endoplasmic reticulum membrane.Mitochondrial matrix proteins can never be mis-targeted to the endoplasmic reticulum during synthesis because MRNAS encoding mitochondrial matrix proteins bind to a special class of cytoplasmic ribosomes that only synthesize mitochondrial proteins they are frequently targeted to the endoplasmic reticulum but are rapidly degraded they don't interact with signal recognition particle they are always synthesized inside the mitochondriaFor each of the following statements regarding protein import into mitochondria, indicate whether it is likely to be true or false. [Select] into the mitochondria matrix. [Select] [Select] being translated [Select] unfolded state. The signal sequence is always retained after proteins are imported Chaperon proteins are needed for protein import into mitochondria ✓ Imported proteins cross the mitochondrial membrane while it is still Proteins imported into mitochondria cross the membrane in
- Determine the reason due to which it is possible that the translation of a single mitochondrial protein might be affected specifically.Explain the reason why mitochondria requires a special translocator to import proteins across the outer membrane, when the membrane already has porins.Describe what would happen to the precursor of a mitochondrial matrix protein in the following types of mitochondrial mutants: (a) a mutation in the Tom22 signal receptor; (b) a mutation in the Tom70 signal receptor; (c) a mutation in the matrix Hsp70; and (d) a mutation in the matrix signal peptidase.
- Using the answer code on the right, indicate which form of energy production is being described: 1. takes place in the mitochondrial matrix 2. produces H2O as a by-product 3. results in a rich yield of ATP 4. takes place in the cytosol 5. processes acetyl-CoA 6. takes place in the mitochondrial innermembrane cristae 7. converts glucose into two pyruvate molecules 8. uses molecular oxygen 9. accomplished by the electron transport system and ATP synthase (a) glycolysis (b) citric acid cycle (c) oxidative phosphorylationExplain a mechanism for how a nuclear-encoded protein is targeted to the inner membrane of the mitochondrion. Explain the mechanism for how a nuclear-encoded protein is targeted to the thylakoid membrane. Describe an experiment that could be conducted to show that there is a targeting system for a nuclear encoded protein that ultimately finds its way to a specific organelle.VDAC1 mitochondrial protein plays an important role in human diseases and many strategies can be taken to utilize the therapeutic potential of VDAC1. what are these diseases and strategies?
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