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Mitochondrial Oxidative Phosphorilation and Genetics Abigail Hardy.

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Presentation on theme: "Mitochondrial Oxidative Phosphorilation and Genetics Abigail Hardy."— Presentation transcript:

1 Mitochondrial Oxidative Phosphorilation and Genetics Abigail Hardy

2 The Mitochondria Thousands of mitochondria in high energy areas Thousands of mitochondria in high energy areas Contact sites facilitate the entry of protiens into matrix Contact sites facilitate the entry of protiens into matrix Produces ~90% of the cellular ATP in eukaryotic cells Produces ~90% of the cellular ATP in eukaryotic cells

3 Major Protien Complexes

4 Oxidative Phosphorylation Complexes I to IV, in addition to other components, act together as an electron transport chain Complexes I to IV, in addition to other components, act together as an electron transport chain Protons in intermembrane space form an electrochemical gradient, which provides potential energy used by complex V for the phosphorylation of ADP to ATP. Protons in intermembrane space form an electrochemical gradient, which provides potential energy used by complex V for the phosphorylation of ADP to ATP. Membrane channel protien (ANT) transports ATP out and ADP into matrix Membrane channel protien (ANT) transports ATP out and ADP into matrix –1:1 ratio of transfer Both ADP and ATP move freely through the outer membrane Both ADP and ATP move freely through the outer membrane

5 Mitochondrial DNA Mitochondria have their own genetic apparatus Mitochondria have their own genetic apparatus Circular (16,568 bp) Circular (16,568 bp) 2-10 separate mitochondrial gemomes in matrix 2-10 separate mitochondrial gemomes in matrix Carries information for: Carries information for: –2 rRNAs –22 tRNAs –13 proteins The 13 proteins encoded are involved in OXPHOS functions The 13 proteins encoded are involved in OXPHOS functions Nuclear genes encode about 70 additional protiens that make up the OXPHOS system Nuclear genes encode about 70 additional protiens that make up the OXPHOS system A mitochondrion is the joint product of two different genetic systems A mitochondrion is the joint product of two different genetic systems

6 Human Mitochondrial Genome

7 Mitochondrial Replication During mitochondrial multiplication each genome is randomly distributed to a daughter mitochondrion During mitochondrial multiplication each genome is randomly distributed to a daughter mitochondrion Does NOT follow the Mendelian pattern of inheritance b/c not part of chromosomal system Does NOT follow the Mendelian pattern of inheritance b/c not part of chromosomal system Details of mitochondrial reproduction are not known Details of mitochondrial reproduction are not known

8 Mitochondrial Genetics Mitochondria of the zygote come from the oocyte (mother) and almost never the sperm (father) Mitochondria of the zygote come from the oocyte (mother) and almost never the sperm (father) If the same set of symptoms in a number of various multigenerational families is inherited only from affected females and almost never from affected males, then there is a strong likelihood that it is caused by a mutation in one of the mitochondrial DNA genes If the same set of symptoms in a number of various multigenerational families is inherited only from affected females and almost never from affected males, then there is a strong likelihood that it is caused by a mutation in one of the mitochondrial DNA genes

9 Oocyte Mitochondria Human oocyte loses mitochondria as it matures through the genetic bottleneck Human oocyte loses mitochondria as it matures through the genetic bottleneck Mitochondria are randomly distributed into daughter cells Mitochondria are randomly distributed into daughter cells Mitochondrial Mutation Load= the ratio of mutated to normal mitochondrial DNA Mitochondrial Mutation Load= the ratio of mutated to normal mitochondrial DNA Homoplasmy= all mitochondria of a cell or tissue have the same genome Homoplasmy= all mitochondria of a cell or tissue have the same genome Heteroplasmy= contains both mutant and wild- type genomes Heteroplasmy= contains both mutant and wild- type genomes –Proportion of mutant to wild-type M. DNA determines if energy shortage occurs Threshold for phenotypic expression Threshold for phenotypic expression

10 Bibliography  Widmaier, E.P., Raff, H., Strang, K.T. Vander’s Human Physiology: The Mechanisms of Body Function. 10 th ed. Boston: McGraw-Hill,  dave/roanoke/bio101ch06.htm dave/roanoke/bio101ch06.htm dave/roanoke/bio101ch06.htm


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