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Organelles
Medical Cell Biology
| Term | Definition |
|---|---|
| Polarized Cells | 2 different cell membrane. Apical BM and Basal Lateral BM |
| Unpolarized Cells | One plasma membrane |
| Double membrane organelles | Mitochondria and Nucleus |
| Secretory pathway | Made in ER - Golgi - Secretory Vesicle - Plasma Membrane |
| Non-secretory Pathway | Made in ER - Golgi - Secretory Vesicle - Endosome - Lysosome (need M6P) |
| Double Membrane - Inner | Contain nuclear lamina (fibrous network of IFs for shaping nuclear envelope) |
| Double Membrane - Outer | Continuous with rough ER |
| Nucleolus | where rRNA synthesized |
| Nuclear Matrix | Inside nucleus, provide scaffold for chromatin binding |
| Nuclear Pores Complex | Transport materials in and out of nucleus. Contain 8 protein subunit. These proteins are joined by spokes. Allow free movement of anything smaller than 9 nm or 60kDa |
| NLS | Protein that helps transport materials IN the nucleus if it is large |
| NES | Protein that helps transport material OUT of the nucleus if is is to large for diffusion |
| Nucleoskeleton | The ring attached inside of the nuclear pore complex |
| Cytoskeleton | The ring attached on the the outside of the nuclear pore complex |
| Nuclear Lamina | Fibrous network of intermediate filaments that underly the inner surface of nuclear membrane. Composed of Lamin A, B,C |
| Prophase | Condense of chromosome by depolymerization and break down of nuclear membrane - through phosphorylation of lamins by lamina kinase |
| Telophase | Decondense of chromosome, reassembly of nuclear envelope by dephosphorylation of lamins by lamin phosphatases |
| Emery-Dreifuss muscular dystrophy | Mutation in Emerin. Elbow and neck become stiff. Cardiac conduction block and weakness |
| Familial partial lipodystrophy | Skin condition. Loss of subcutaneous fat |
| Charcot marie tooth disorder type 2 | Non-demyellinating peripheral neuropathy, Distal muscle weakness and atrophy |
| Hutchison Gilford Progeria | Premature aging |
| Functions of SER | Glucose mobilization from glycogen, Storage of calcium, Drug detoxification, lipid synthesis |
| Glucose-6-Phosphatase | An enzyme that breaks glycogen into glucose. Remove Pi group in order to export |
| Von Gierke's Disease | Increase glycogen storage cause hypoglycemia, enlarged liver and kidney, abnormal growth |
| Cytochrome p450s | Use oxygen and NADPH to hydroxylate steroids and drugs |
| Hydroxylation | Means increase water solubility to hydrophobic drugs |
| Chronic barbiturate use | Increase in p450 and SER and SER expansion |
| Lipid Synthesis: Phospholipids | Assembly in SER and flippase will distribute |
| rER function | Glycosylation, folding, quality control, and degredation of proteins not passing quality control |
| Signal Recognition Particle (SRP) | Direct ribosome and mRNA to rER membrane. Nascent polypetide translocated across ER membrane. |
| Signal Peptidase | Cleaves signal sequence |
| P54 | Binds signal peptide |
| P9/P14 | Binds ribosome carrying mRNA |
| P68/P72 | Needed for translocation |
| P19 | Attaches p54 to the rest of the SRP |
| What is needed for integral membrane proteins translation and translocation | Signal Sequence and Stop Transfer sequence |
| Type I Integral Membrane Protein | Has an N-term cleavalbe signal peptide and stop transfer anchor |
| Type II Integral Membrane Protein | No cleavable peptide site, positive N-terminal side |
| Type III Integral Membrane Protein | No cleavable peptide side, positive C-terminal side |
| Type IV Protein | Multi stop transfer sequence and signal anchor |
| Mannose-6-phophate (M6P) | During protein glycosylation, Mannose-6-phosphate is added to target lysosomal enzymes to lysosome |
| N-Linked glycosylation | Occurs in the rER. A tree of sugars (oligosaccharide) is added to Asn residues |
| Chaperones | Needed for proper folding |
| ERAD | Misfolded proteins will be sent to ERAD (ER associated degradation pathway) |
| BiP | Shield hydrophobic residues inside |
| Protein Disulfide isomerases | Form disulfide bond b/w cysteine and sulfhydryl group |
| Calreticulin & Calnexin | Bind glucose on N-Linked oligosaccharides to promote folding |
| Calnexin | Single pass TM protein, need to bind to ERp57 for proper folding |
| Calreticulin | Soluble protein |
| Cystic Fibrosis | Mutation in CFTR, deletion of phenylalanine at Position 508, Protein misfolded-- send to degradation, Defective Cl channel, Cl cannot taken inside the cell |
| Scaffold proteins of coat | Determined what cargo is selected |
| Formation of Vesicle Coat | First recruitment of small GTP-binding protein that is initiated by GEF |
| Vesicle Tethering | Regulated by Rab |
| Choroideremia | Defective Rab, vision disorder |
| Vesicle Fusion | Need SNARES, V-snares and T-snares |
| NSF (N-ethylmaleimide sensitive factor) | ATPase that disassemble SNARE complexes |
| COP II | Anterograde transport. ER to Golgi |
| Sar1 | Recruits COPII |
| Sec 23 and Sec 24 | Adaptor protein, inner layer for COP II coat |
| Sec 13 and Sec 31 | Adaptor protein, outer layer for COP II coat. |
| ERGIC53 | Escort protein for COPII anterograde transport |
| Mutation in ERGIC53 | Cause bleeding disorder FACTOR V and VIII |
| O-linked glycosylation | Takes place by glycosyl transferases. Occurs in the golgi |
| COP I | Retrograde transport. Golgi to ER. Return SNARE proteins to rER |
| KDEL | C-terminal retrograde transport signals. Soluble protein. Needed in COP I. |
| KKXX | C-terminal retrograde transport signal. Nonsoluble, hydrophobic membrane proteins, Needed in COP I. |
| Regulated Secretion | Store proteins and peptides-release when needed. N-glycosylation |
| Constitutive Secretion | Replinish (fill or restore) lipids and proteins, no coat protein idenitified. |
| I-Cell Disease | Defect in the GlcNac phophotransferase. Lysosomal enyzme is not phosphorylated and secreted so will get accumulation of undigested glycolipids |
| Clathrin Coat | TGN to Lysosome |
| ARF-1 | Recruit clathrin to bind to vesicles form a coating |
| AP-1 | adaptor proteins is in inner layer of the coat that bind to cargo protein |
| Cholesterol and LDL Receptor | Inner coat layer is AP-2, LDL bind Apo-B 100 then bind to LDL receptor then bind to AP-2 formed a coat vesicle |
| Transferrin | Iron binding blood plasma glycoprotein. Low pH induce conformational change that stimulates the release of iron |
| Proteasome | Needs ubiquitin to attach to target |
| Ubiquination | Needed for translation, activation of transcription DNA repair, apoptosis |
| Ring E3 | Direct transfer |
| HECT E3 | Indirect Ub transfer to target |
| E4 | Conjugating factor for polyubiquitination |
| Ubiquination complex | Consist of 2alpha (outer) and 2beta (inner). Consists of two 19S core and one 20S core unite |
| 19S | Recognition of target |
| 20S | Degradation of target |
| Functions of Mitochondria | ATP production, Kreb Cycle, Heme synthesis, gluconeogenesis, Regulators of programmed cell death |
| MLS (Mitochondrial localization signals) | Needed for protein transport |
| Cardiolipin | Phospholipid that is located in the inner membrane of a mitochondria |
| Mitochondrial Genome | 2rRNA genes, 22tRNA genes, 13 genes for electron transport chain and oxidative phosphorylation |
| Mitochondrial Diseases | Maternally inherited |
| LHON (Leber's Hereditary Optic Neuropathy) | Bilateral, point mutations in mitochondrial DNA (ND1, ND4, ND6) subunit of NADH-ubiquinone Oxidoreductase |
| Protein Import: Matrix Protein | Binds to TOM 20/22-- Goes through TOM 40 -- Transferred to TIM 23/17 complex and passes through TIM 50 -- PAM |
| Protein Import: Outer Membrane | Binds to TOM 20 -- Passes through TOM 40 -- Then through TIMS-- TIMS bring it to SAM 50 -- Becomes beta barrel protein |
| Protein Import: Inner Membrane | Carrier Portein precursor binds to TOM 70 -- Translocate through TOM 40 General -- Present it to small TIMS in the inter membrane space -- Small TIMs present it to TIM 22 complex -- Now get folded and become carrier proteins |
| Oxidase | Peroxisome protein that is involve in hydrogen peroxide catabolism |
| Catalases | Peroxisome protein that is involved in hydrogen peroxide breakdown |
| Functions of Peroxisome | VLC fatty acid oxidation, Cholesterol and bile synthesis, Synthesis of Plasmologens which is synthesis of Ether Lipids by ester linkage (R'-O-R), Detoxification of H2O2. |
| Import of peroxisomal proteins | Must be FOLDED, Need Peroxisomal localization Signal |
| PTS1 | C terminal target sequence (Peroxisome) |
| PTS2 | N terminal target sequence |
| PEX5P | Cytosolic receptor of PTS1 |
| PEX7P | Cytosolic receptor of PTS2 |
| Are PTS removed inside the peroxisome | NOOOO! |
| X-linked adreno leukodystrophy (X-ALD) | Peroxisomal Disease, Accumulation of VLC acid in brain and adrenal cortex, due to defective membrane transport protein so cannot transport inside the peroxisome, Destroy myelin sheath around the nerves cell |
| Zellweger Syndrome | Defect in PEX receptor, inability to import of peroxisomal proteins and enzymes |
Created by:
jsabangan
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