ENERGY DEFICIENCY IN ORGANIC ACIDEMIAS. Kimberly A. Chapman, M.D., Ph.D. Children s National Medical Center
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1 ENERGY DEFICIENCY IN ORGANIC ACIDEMIAS Kimberly A. Chapman, M.D., Ph.D. Children s National Medical Center 1
2 What is energy? Energy is the money that drives all biological processes Protein (amino acids), carbohydrates (CHOs), fatty acids 2
3 Big Energy Users Growth Eye (retinas) Brain: Structure and function Heart Kidneys Muscles Wajner
4 Energy phenotype Eye (retinas) Growth Brain: Structure and function 20-25% Heart: 9% Kidneys Muscles Wajner
5 Complex 5
6 PA and MMA V O M I T Propionyl CoA Methylmalonyl CoA Succinyl CoA 6
7 PA and MMA V O M I T Propionyl CoA Methylmalonyl CoA Succinyl CoA 7
8 What happens if block 8
9 PA and MMA V O M I T Propionyl CoA Methylmalonyl CoA Succinyl CoA Methylcitrate 3-hydroxypropinate 9
10 PA and MMA V O M I T Propionyl CoA Methylcitrate 3-hydroxypropinate Methylmalonyl CoA Methylmalonic Acid Succinyl CoA 10
11 Energy makes AcetylCoA Oxaloacetate Citrate Malate Fumarate Succinate Succinyl CoA Isocitrate α-ketoglutarate Methylmalonyl CoA Propionyl CoA 11
12 12
13 PA/MMA effects AcetylCoA Oxaloacetate Malate Fumarate Succinate Succinyl CoA Methylmalonyl CoA Propionyl CoA Citrate Isocitrate α-ketoglutarate 13
14 How do you know this? C. elegans who missing pcca or pccb accumulate citrate and have deficient malate compared to controls (submitted) AcetylCoA Oxaloacetate Malate Fumarate Succinate Citrate Isocitrate a-ketoglutarate SuccinylCoA MUT MethylmalonylCoA PCC 14
15 With time.we think AcetylCoA Oxaloacetate Malate Fumarate Succinate Succinyl CoA Citrate Isocitrate α-ketoglutarate Methylmalonyl CoA Propionyl CoA 15
16 Why not significant at birth? Other systems to make energy (multiple generators) Cell may compensate with time (changing normal balances of other things to make up) More responsibilities as get older 16
17 As age More demand Functional New Construction Energy for repair 17
18 But those generators and side reactions get old 18
19 Slowing to very slow and stopping AcetylCoA Oxaloacetate Malate Fumarate Succinate Succinyl CoA Citrate Isocitrate α-ketoglutarate Methylmalonyl CoA Propionyl CoA 19
20 Energy Presentations Metabolic Strokes Renal dysfunction Cardiomyopathy and Long QTc Optic Atrophy Myopathy Elevations of Lactate Increased free radical products in urine 20
21 Now what? 21
22 Tasks for doing Measuring energy needs accurately Are there ways to work around the block (using alternative pathways)? 22
23 Approaches to doing Measuring Energy Needs accurately Resting Metabolic Rate (RMR) Hauser et al. published in 2010 that individuals with MMA have about 20% less energy than predicted by math 23
24 Alternative pathways Leucine AcetylCoA Oxaloacetate Citrate Malate Leucine Fumarate Succinate Succinyl CoA Isocitrate α-ketoglutarate Glutamine/glutamate Methylmalonyl CoA Propionyl CoA 24
25 Leucine does not kill PA cells 9 year old female with PA D1 D3 0 RMPI NO GLU MEM ASP LEU 25
26 Free Radical release Glutathione levels are down In MMA Urine F-2-isoprostanes (lipid oxidation) di-tyrosine (protein oxidation) increased especially Cbl B, and Cbl C 1 L-carnitine decreases above elevation 2 1 McGuire Ribas
27 Can we use antioxidants to better protect the mitochondria? Vitamin E and C CoEnzyme Q Thiamine (B vitamins) Gamma-glutamyl-cysteine N-acetylcysteine L-carnitine Others 27
28 Should I do these thing now? No Clinical trials are really important We don t know if any of these things will help We need good markers if they are working (like RMR) Trial designs are in preparation 28
29 What can I do for my child? Maximize nutrition Wash hands (prevent infection) Flu shots and immunizations 29
30 Questions and Discussion 30
31 Acknowledgements CNMC Marshall Summar Gary Cunningham Maddie Shankle Ariel Markowitz-Shulman Funding NICHD (CHRCDA) program (05K12- HD ) Research studies funded in part by PA foundation. CHOP Marni Falk Zhe (Jim) Zhang Meera Rao Julian Ostrovsky Evgueni Daikhin Ilana Nissim Marc Yudkoff 31
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