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24 Cards in this Set
- Front
- Back
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Enzymes in Peroxisome
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Oxidases
Catalase β-Oxidation enzymes Enzymes for biosynthesis of cholesterol, bile acids, plasmalogens |
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Peroxisomal Oxidase Rxn
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O2-dependent oxidation, produce H2O2
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Peroxisomal Catalase Rxn
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Detoxification:
- Convert H2O2 to H2O + O2 Or - Use H2O2 to oxidize alcohol/nitrates in liver |
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Peroxisomal β-Ox'dn vs Mitochondrial β-Ox'dn
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Long & very long lipid
vs Short lipid |
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Peroxisome Formation
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Growth and fission of existing Prxm
*some de novo biogenesis |
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Peroxisomal proteins formation
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All encoded by nucleus
Import into Prxm directed by PTS (Prxm Targeting Sequences) or mPTS to matrix or membrane |
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Zellweger's Syndrome
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Protein import disorder during Prxm biogeneis
*linked to craniofacial defects |
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X-ALD
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- X-linked Adrenoleukodystrophy
- Defects in Prxm Transporter Protein (involed in VLCFAs breakdown) - VLCFA accum., systemic dmg - Lorenzo's oil to replace plasmoalogens |
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Mitochondrial Compartment Enzymes
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MOM - Porin
IMS - Enzymes that +(P) nucleotides using ATP MIM - ETC enzymes and ATP synthase Matrix - enzymes for β-ox'dn, pyruvate ox'dn, TCA |
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Principal roles of Mitochondria
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- Make ATP thru TCA and OxPh
- Tissue homeostatis (role in apoptosis) - Make ROS - Calcium homeostasis - Steroid and heme synthesis |
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How to make ATP production spontaneous?
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Couple ADP +(P) (7.3kcal)
with NADH ox'dn (-37.8kcal) and O2/H+ reduction (-14.8kcal) |
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3 Major steps to extract chemical E from glucose
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1. Glycolysis
2. TCA 3. Oxidative +(P) |
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Glycolysis Summary
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2 (Pyruvate + NADH + ATP + H+ + H2O)
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TCA Summary
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GTP + FADH2 + 3 NADH + 2 CO2 + 2 H+ +CoA-SH
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Where does TCA take place?
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Matrix
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Products of TCA
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GTP, FADH2, 3 NADH
Releases CO2 |
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PMF
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Proton-Motive Force
- the electrochemical potential of protons across MIM *depends on Temp and pH *5 kcal for each proton |
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How much to make one ATP under cellular conditions?
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about 10 kcal according to Peter Mitchell's Chemiosotic hypothesis
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Endosymbiotic hypothesis
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Origin of mitochondria
Aerobic bacteria ingested by anaerobic bacteria, survival advantage, symbiotic partnership |
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mtDNA
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Circular dsDNA plasmid
No histones 10x higher mutation rate |
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# genes and products on human mtDNA
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- 37 genes
- 13 proteins, 2 rRNA, 22 tRNA |
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mtDNA inheritance
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Maternal
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How does MT import proteins
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- targeting sequence
- TOM (Outer Membrane) and TIM (Inner Membrane) membrane proteins |
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Mitochondrial link to Neurodegeneration
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PD, Alz, Hunt - MT dynamics problems
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