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13 Cards in this Set
- Front
- Back
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Describe what is found in the:
1. OMM 2. IMS 3. IMM 4. Matrix (p32-3) |
1. Voltage-dependent anion channels, receptors, translocases of OMM
2. Cytochrome C, adenylate kinase 3. Cardiolipin (lipid key to metabolism), ETC enzymes, ATP-ADP translocase, translocases of IMM 4. Kreb cycle enzymes, peptidases, mtDNA, Ribosomes, Ca++ |
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What is the charge in the matrix?
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Negative
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mtDNA encodes for all of the ETC enzymes except:
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complex II (succinate-CoQ reductase)
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What does the nuclear genome contribute to mitochondria?
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1. Mitochondrial matrix enzymes
2. Cooperates with mt genome in assembly of multisubunit enzyme complexes of oxidative phosph 3. Encode components of protein (and small RNA) import machinery 4. TFs for mtDNA transcription and replication and protein that target mt (ie. controls translation of specific mt mRNAs) 5. Controls processing of specific mRNA precursors |
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What percentage of mt proteins are imported? Describe targeting sequence that gets them there.
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90%. A leader signal sequence 20-70 aa's long, rich in basic and hydroxylated aas, usually at N-terminus, can form amphipathic structures, and usually cleaved by peptidases in mt.
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Be sure to review p35 of notes and p16 of lecture notes CTB_3_2012. No NCs were made for this.
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Be sure to review p35 of notes and p16 of lecture notes CTB_3_2012. No NCs were made for this.
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What are the three possible sources of mutation in mtDNA?
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1. Nuclear DNA: mendelian inheritance
2. Cytoplasmic mtDNA: non-mendelian; maternal inheritance 3. X-linked |
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Why is mtDNA so susceptible to mutation? (3)
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1. mtDNA is attached to IMM, source of ROS
2. mtDNA lacks protective histones 3. mtDNA has limited repair system. |
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What is LHON, what are symptoms, and what causes it?
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Leber's Hereditary Optic Neuropathy is a missense mutation that results in an amino acid substitution. Affects pt overnight in one eye (blindness); 2nd eye later.
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What are the two biogenesis mutations discussed in class. What causes them (2)
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MERRF and MELAS (Myoclonus Epilepsy and Ragged Red Fibers; Mitochondrial Encephalomyopathy, Lactic Acidosis, & Stroke-like symptoms). Caused by tRNA point mutations that affect protein synthesis.
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What are the two mt diseases caused by insertion-deletion mutations?
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KSS (Kearns Sayre Syndrom) and PEO (progressive External ophthalmoplegia)
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Disease of aging cause what things to happen in the mitochnondria?
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1. reactive oxygen species damage mtDNA
2. mtDNA deletions accumulate with age 3. Oxidative phosphorylation declines with age. |
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Overall: you may want to review this lecture for details that were not included in the nc's you made
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Overall: you may want to review this lecture for details that were not included in the nc's you made
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