复旦大学：《医学遗传学 Medical Genetics》课程教学资源（PPT课件讲稿）07 线粒体疾病的遗传 Inheritance of Mitochondrail Diseases

Medical Genetics 07线粒体疾病的遗传 Inheritance of Mitochondrail diseases

Medical Genetics 07线粒体疾病的遗传 Inheritance of Mitochondrail Diseases

Medical Genetics The normal 46 chromosomes in our body are contained in the center of the cell which is called the nucleus, mitochondria are structures in the cell located outside of the nucleus in the cytoplasm that also contain genes that are separate from the ones in the nucleus

Medical Genetics The normal 46 chromosomes in our body are contained in the center of the cell, which is called the nucleus. Mitochondria are structures in the cell located outside of the nucleus in the cytoplasm, that also contain genes that are separate from the ones in the nucleus

Medical Genetics Mitochondria are extremely small (from.002-008 millimeters in length) rice-shaped structures whose details can only be seen with the electron microscope

Medical Genetics Mitochondria are extremely small (from .002-.008 millimeters in length) rice-shaped structures whose details can only be seen with the electron microscope

Medical Genetics Mitochondria are often called the powerhouses"of the cell because they are the site where sugar is broken down to release the energy required for cellular functions

Medical Genetics Mitochondria are often called the “powerhouses” of the cell because they are the site where sugar is broken down to release the energy required for cellular functions

Medical Genetics Cell Chromosome Nucleus Golgi apparatus Mitochrondrion 0D Ribosomes Endoplastic reticulum

Medical Genetics

Medical Genetics a cell may have hundreds or even thousands of mitochondria depending on the particular cell's need for energy.(For example, the average human liver cell contains more than a thousand mitochondria.)

Medical Genetics A cell may have hundreds or even thousands of mitochondria depending on the particular cell’s need for energy. (For example, the average human liver cell contains more than a thousand mitochondria.)

Medical Genetics In addition to containing the many proteins needed to control the energy release process, the mitochondria also contain a small amount of dna that is used to direct the manufacture of thirteen of the proteins needed for its activities

Medical Genetics In addition to containing the many proteins needed to control the energy release process, the mitochondria also contain a small amount of DNA that is used to direct the manufacture of thirteen of the proteins needed for its activities

Medical Genetics DNA Aminoacyl tRNA polymerase Initiation factors synthetases Replication Transcription tRNAs DNA mRNAs Translation Respiratory proteins TRNAs RNA Ribosomal Other respiratory polymerase proteins proteins

Medical Genetics

Medical Genetics Unlike nuclear genes, which are inherited from both parents, mitochondrial genes are inherited only from the mother

Medical Genetics Unlike nuclear genes, which are inherited from both parents, mitochondrial genes are inherited only from the mother

Medical Genetics If there is a mutation in a mitochondrial gene it is passed from a mother to all of her children sons will not pass it on, but daughters will pass it on to all of their children, and So on

Medical Genetics If there is a mutation in a mitochondrial gene, it is passed from a mother to all of her children; sons will not pass it on, but daughters will pass it on to all of their children, and so on