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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- In the mitochondrial disease MNGIE a deficiency of thymidine phosphorylase causes dTTP to accumulate in mitochondria. Describe the mechanism by which this occurs. A secondary effect is depletion of mitochondrial dCTP pools. Describe a plausible mechanism for this effect.Although the outer mitochondrial membrane is permeable to all small molecules, the inner mitochondrial membrane is essentially impermeable in the absence of specific transport proteins. Consider this information answer: Present two types of benefits derived from separating the reactions of glycolysis in the cytosol from those that occur during the citric acid cycle in the mitochondrion.A mitochondrial matrix protein is maintained in its unfolded state by a chaperone protein before transport across the mitochondrial membrane true false
- 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.In the presence of oxygen, the mitochondrion in yeast is used for aerobic respiration,however, under anaerobic conditions,the yeast mitochondria have been found to have other function. Identify and briefly discuss four of these functionsOnly 37 of the proteins in mitochondria are encoded by the mitochondrial DNA. The other proteins are encoded in nuclear DNA, then translated and transported to the mitochondria. Describe one unique feature of protein transport across mitochondrial membranes.
- Uncoupling agents are proteins spanning the inner mitochondrial membrane that allow protons to pass through the membrane and bypass the channel of ATP synthase. Describe the consequences to the proton gradient and ATP production.?What role does cellular respiration play in mitochondrial diseases.An important function of the inner mitochondrial membrane is to provide a selectively permeable barrier to the movement of water soluble molecules and thus to generate different chemical environments on either side of the mem- brane. However, many of the substrates and products of oxidative phosphorylation are water soluble and must cross the inner membrane. How does this transport occur?
- 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?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?Citrate synthase is a nuclear-encoded enzyme of the Citric Acid Cycle. The enzyme is functional in the mitochondrial matrix. Looking at the system required for mitochondrial targeting and import of citrate synthase, which of the following statements are correct? 1. Proper import and processing of citrate synthase is dependent upon other proteins found in the mitochondria. 2. Because of the signal peptide, the mature functional enzyme is actually longer than it would be if it was coded by the organelle genome. 3. Because the citrate synthase signal peptide doesn’t have a precise amino acid signature, mutations that slightly change the primary sequence have no effect on targeting. 4. To get citrate synthase into the mitochondrial matrix requires two signal sequences: one to get it across the outer membrane and a second to get it across the inner membrane. A. 1,2 and 3 B. 1 and 3 C. 2 and 4 D. 4 only E. All of 1,2,3 and 4 are correct