复旦大学上海医学院：《医学遗传学 Medical Genetics》课程PPT教学课件（英文）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

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 powerhousesof 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 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 smal 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 factor synthetases Replication Transcription tRNAs DNA mRNAs Translation Respiratory proteins TRNAs RNA Ribosomal Other respirator polymerase proteins proteins

Medical Genetics

Medical genetics Unlike nuclear genes, which are nherited 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