NS 3410 1st Edition Lecture 2Outline of Last LectureI. ObjectivesII. SyllabusIII. GradingOutline of Current LectureI. TeratomaII. RibosomopathiesIII. Endoplasmic Reticulum Associated Destruction- ERADIV. MitochondriaCurrent LectureI. Teratoma- Kind of tumor with tissue or organ components resembling normal derivatives of all three germ layers (ectoderm, mesoderm, endoderm)- Can contain hair, teeth, bone and sometimes complex organs- It is encapsulated, so usually benignII. Ribosomopathies- Ribosomes generate all cellular proteins needed for cell growth-translate mRNA into protein- Mutation that leads to complete loss often lethal- Defects in single ribosomal protein or ribosome assembly factors can affect specific cell types and cause tissue-specific human diseasesThese notes represent a detailed interpretation of the professor’s lecture. GradeBuddy is best used as a supplement to your own notes, not as a substitute.- Human diseases caused by ribosomopathies-Diamond-Blackfan anemia-Congenital asplenia-Treacher-Collins syndrome- Not all ribosomes are created equally- Get very different phenotypes/diseases from different types of ribosomal mutations- Composition of ribosomes may be different in different cell types- Cell environment can cause ribosome to have preference for translating specific types of mRNAs- Shared characteristics of these diseases include:-Small stature-Microcephaly-Hematological defects-Predisposition to cancerIII. Endoplasmic Reticulum Associated Destruction-ERAD- Monitors folding of membrane and secretory proteins, directs mis-folded proteins to proteasome for degradation- Works to control the turnover of specific proteins to achieve a certain physiological state- Regulated degradation process- Accumulation of mis-folded proteins leads to ER stress- ER stress due to:-Ischemia-Glucose/nutrient deprivation-Viral infections-Excess lipids-Human disordersIV. Mitochondria- Mitochondria have their own DNA- Only in egg so only inherit from mother- Contains 37 genes- Within a cell, all mitochondria can have identical mtDNA (homoplasy) or contain 2 or more mitochondrial genomes (heteroplasmy)- Damage causes neuronal dysfunction, cell death and movement