Cell Tissue & Organ Biology Exam 1

Front 1

Emergence

Back 1

"new properties emerge @ each higher level of organization that could not have been predicted from knowledge of lower-level components"
- Ernst Mayr

ex. H20 -- or the reason we study CTO Bio

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RBC Scale

Back 2

10 μm across

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Progeria

Back 3

rapid aging disease

developmentally like a kid (mental, no puberty), but appear old, die of cardiac dis.
*Laminopathy

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Huntington's

Back 4

norm: huntingtin -- cytoplasmic prot

dis: prot. accumulates in nucleus, disturbs fxn

3 dimensional stuc (w. added repeat seq) acquires a nuclear localization sequence (NLS)

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Lupus

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autoimmune

autoantibodies complex w/ nuclear targets → destroying cell

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Chormatin

Back 6

nuclear DNA / prot complex

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Heterochromatin

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↑ condensed, transcriptionally inactive chromating (during interphase)

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Euchromatin

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less condensed (diffuse), transcriptionally active chromatin (during interphase)

represents active gene transc.

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Nuclear Envelope

Back 9

2 membranes separated by perinuclear space (20 nm)

Nuclear pores join membranes (d= 80 nm)

outer: ribosomes attaches, continuous w/ Rough & Smooth ER

Inner: assoc. w/ fibrous lamina

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Nuclear Lamina

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50 nm layer of fibrous proteins (nuclear lamins)

assoc. w/ inner nuc membrane and pore complexes.

*structural stability
*anchor site for pore complexes and peripheral chromatin
*regulates disassembly of nuc envelope during mitosis

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Nuclear Pore Complexes

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assoc. w/ nuc pore, can constrict opening to <10-15 nm

8 prot subuints form annuli (rnigs) on both sides of nuc envelope connected by central diaphragm

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Nuclear Matrix

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complex of fibrillar prot.

a site of RNA processing

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Nucleolus

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1+ / nucleus

ribosome-producing machines

loops of DNA from several chromosomes w/ clusters of ribosomal RNA genes

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Ribonucleoprotein Complex

Back 14

RNA + RNA binding prot. necessary to assemble ribosome

made in nucleolus and transp. to cyto
*prevents transc. by fxn ribosome in nucleus

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Laminopathies

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*Progeria
*Lipodystrophy
Cardiomyopathy (rare form)
Musc. Dystrophy (rare form)
Aytpical Werner's Syndrome
Mandibular Aeryldrsplasia
Restrictive Dermopathy

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Restrictive Dermopathy

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baby born, skin doesn't grow

as organs grow → dies a very painful death

Front 17

Nuclear Transport Mechanisms

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1. nuclear pores are entry sites

2. specific a.a. seq. targets prot. to nuc.

*ATP required for transport, not binding & recognition

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NLS

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nuclear localization sequences

no single consensus seq for nuc. localization

freq. importin (60 kDa) binds NLS, req. for import

Front 19

Defective Nuclear Transport Diseases

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Huntington's
Lupus

Front 20

Nuclear Assembly-Disassembly

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1) lamins connect chromatin to nuc. memb.
2) lamins unphosphorylated in interphase
3)early mitosis, lamins phosphorylated by a kinase, case chromatin-membrane connection to break → disassembly
4)late mitosis, phosphatase dephosphorylates lamins, → permits nuc. mem. assembly

Front 21

Membranes

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Plasma: forms boundary between cell and external environment

Cytoplasmic: separate cytoplasm from organelle interior (lumen) ex. ER, golgi

*Separate dissimilar environments

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General Membrane Functions

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1) Protection

2) Permeability Regulation

3) Communication

4) Organization

*deformability and selectivity of substances transported

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Classes of Lipids

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1. Phospholipids

2. Sphingolipids

3. Eicosanoids -- fxn import (not struct)

4. Cholesterol

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Lipid Structure

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asymmetric spontaneous fluid bilayer
(flexibility from acyl chain, head groups give order)

fluidity α temp, chol. content, degree of unsaturation

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Membrane Proteins

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1. Integral -- only extracted by dissolving entire membrane (receptors)

2. Peripheral -- can be removed w/ milder treatments (pH, ionic strength)
ex. spectrin, gibronectin

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Eicosanoids

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Protoglanids -- induce labor, vaso dil / constric, smooth musc. contract.

Thromboxanes -- platelets

Leukotrienes -- immunology, vaso dil. bronchioconstriction

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Advair

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salmeterol stimulates beta2 adrenergic receptors, relaxes smooth muscules in airway

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Membrane Protein Structure

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1. Tri-domain structure: extracellular, mem. spanning, intracellular

2. extracellular glycosylated

3. may span more than once.
4. may contain more than one subunit

Front 29

Membrane

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50% lipid
50% protein
some CHO

ex. mito IM 76% prot., 24% lipid
ox. phos, conduct
myelin 18% prot, 80% lipid --> insulate

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Thin section electron microscopy

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trilaminar unit membrane approx. 7 nm width appears as two dark lines separated by an electron lucent zone

Front 31

Freeze Fracture Electron Microscopy

Back 31

fracturing frozen tissues splits membranes through hypdrophobic interior at junction between fatty acyl chains of bilayer

can see asymmetric dist. of globular particles -- denser on protoplasmic (P) face than ectoplasmic (E) face

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Permeability

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passive -- ionic diffusion

coupled transport -- ionic exchange

active transport -- sodium pump

charged pores -- channels and gates

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membrane stability

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forces responsible for maintaining integrity of membrane largely based on intermolecular interactions

Front 34

Band 3 Protein

Back 34

exquisite design, anion exchange

provides combination of flexibility and strength

spectrin "cables" run beneath cyto side of mem.

Front 35

Hereditary Spherocytosis

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(assoc w/ rarer Hereditary Elliptocytosis)

mut. prot prevents proper membrane-cytoskeletal interactions
∴ mimsshapen RBCs back up in spleen --> splenomegaly, anemia

autosomal dom.

Front 36

HIV fusion

Back 36

viral coat protein recognized by CD4 (T-cell receptor)

binding --> rapid fusion and entry of viral capsid

viral glycoprot. in infected ell plasma membrane -- can fuse with other CD4+ cells (even w/out virus) --> multinucleated syncytium

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Toxoplasmosis

Back 37

catys carry ↑ [] protazoa, can cause problems in pregnant women

Front 38

Self-Recognition

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mediated by glycoproteins

ex. blood type: O, A, B, AB

Front 39

Coccidiosis

Back 39

rec. spec. CHO on intestinal epithelial surface

Δ strain, Δ CHO rec. further down gut, Δ sources
∴ can diag. type of infection by location

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Mycoplasma

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--> "walking pneumonia"

mycoplasma attach to base of cilia, suck chol. out of membrane for self
--> ↑ fluidity, cilia cannot "beat", ↑ mucous levels

Front 41

Fabry's Disease

Back 41

α-Galactosidase A deficiency

ceramide accumulates

1st disease sucess. treat. w/ enzyme replacement therapy

Front 42

Lysosomal Storage Diseases

Back 42

we know causes & enzyme deficiencies, but hard to treat (delivery problem)

Front 43

Sphingolipids

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similar to phopholipids in _space_

Δ head group, ↑ P (attach CHO)

Front 44

Transport Functions

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1) Regulation of cell volume / maintain pH and ionic gradients

2) uptake of nutrients / excretion of waste

3) development of ionic gradients (excitability)

4) uptake and degradation of pathogenic organisms

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Passive Transport

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down a concentration gradient / does not require energy

spontaneous

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Uniport

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movement of one molecule/ion down electrochemical gradient

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Co-transport

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movement of 2 mol, 1 going down gradient drives movement of the other going against it's gradient

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Symport

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co-transport; both molecules move in same direction

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Antiport

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co-transport; both molecules move in opposite directions

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Active transport

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requires cellular metabolic energy

transport up chemical or electrical gradients

ex. Na+/K+ ATPase (K+ IN, Na+ OUT)
CFTR (Cystic Fibrosis Transmem. Reg.)

Front 51

Tangier Disease

Back 51

rare defect in an ABC ATPase

lack HDL in plasma
develop premature cardiovascular disease and die in 40s/50s
*swollen, bright orange tonsils

*studying has revealed pathway of reverse chol. trans. and HDL role

Front 52

Endocytosis

Back 52

cells retrieve portions of plasma membrane as vessicle

recycles membrane, uptake of solutes from extracellular space, uptake of foreign objects (bac, viruses, fibers)

selective uptake of nutrients (ex. LDL) or ligands (ex. hormones, growth factors)

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Endocytosis steps

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1. Membrane invagination / evagniation
2. Membrane fission (pinching off)
3. Acidification of vesicle
4. Fusion of endocytic vesicle w/ target

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Pinocytosis

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"fluid-phase endocytosis"
*cell drinking

small vesicle (50-70 nm) takes up solutes and non-adsorbed prot.

abnormal fxn implicated in disruption of blood-brain barrier after stroke

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Membrane Permeability

Back 55

Ions and polar molecules don't cross

Front 56

ABC ATPase-Mediated Drug Resistance

Back 56

major mech. of cancer tumor drug resistance

small percentage of tumor cells don't die, ↑ [P glycoprot], pumps drug out

∴ must find new drug target

Front 57

Receptor-mediated endocytosis

Back 57

proteins/viral particles bind specific receptors of cell surf (often in regions w/ clathrin)

selective uptake, concentration, process. nut & cell modulating mol., viral degredation

Front 58

Clathrin

Back 58

extrinsic/peripheral mem. prot. that concentrates ligand-receptor complexes

aids in membrane fission

Front 59

Dynamin

Back 59

prot. req. for pinching off small (50-70nm) vesicle/endosome from membrane

Front 60

Adaptins

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link clathrin to membrane

Front 61

CURL

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Compartment for Uncoupling of Receptor and Ligand

ligand separates from vesicle, generally degraded

Front 62

Phagocytosis

Back 62

bacteria and bigger

*receptor mediated

membrane evagniates to engulf particle, forms phagosome (100-150nm), acidifies by H+-ATPase,

fuses w/ lysosome --> phagolysosome

*internalization and degradation of large objects (freq. at site of inflammation)

Front 63

Diseases of Phagocytosis

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Legionnaire's Disease
Streptococcus
Tuberculosis
Leprosy
Leishmaniasis
Toxoplasmosis/Coccidiosis

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Familial Hypercholesterolemia

Back 64

rare AD dis. -- homozygotes have ↑↑ chol. levels

freq. die of cardiovascular problems before 20.

from failure of LDL to bind receptor, failure of receptor to cluster to clathrin pits, OR receptor missing membrane spanning section
*inability to transport chol.!

Front 65

Streptococcus (exploiting phagocytosis)

Back 65

CHO coat on cell surface NOT recognized as foreign by macrophages or other immune cells

Front 66

Legionnaire's Disease

Back 66

L. pneumophila is phagocytosed by epithelial cells in resp. tract.

rapidly neutralizes acidification process, ∴ ---| fusion of phagosome / lysosome
then, --> proliferation, infection

*{similar mech, Leishmani & leprosy}

Front 67

Pneumonoultramicroscopicsilicovolcanoneosis

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"black lung disease" presenting in coal miners inhaling silica fibers

Front 68

Diseases from inability to degrade inert particles

Back 68

inhalation of asbestosis --> endothelioma (rare, highly malignant cancer)

Front 69

Cytosolic Ribosome Targets

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1. Cytosol (ex. globin)

2. Mitochondria (ex. resp. chain prot.)

3. Nucleus (ex. histones)

4. Peroxisomes

Front 70

RER Protein Targets

Back 70

1. Secretory prot (constitutively prod & secreted or packaged into storage granules and secreted on stimulation)

2. Plasma membrane prot.

3. lysosomal enzymes

4. RER, Golgi, vesicular mem. prot.

Front 71

Chaperone Prot

Back 71

as prot is being synthesized, recognize misfold, ↑ efficiency defective prot. destruction

Front 72

Lysosomal Targeting

Back 72

"high mannose" N-glycosylated glycoproteins, synth in RER, glycosylated in RER/Golgi, some phosphorylated

targeting mediated by phospho-mannose groups w/ Golgi receptor

*sugar struc. added w/ addition and cleavage; specificity of CHO come from ↑ # poss. additions / cleavages

Front 73

Mucopolysaccharidoses

Back 73

lysosome engorged w/ mucopolysaccharides ("glycosaminoglycans")

Front 74

I-cell disease

Back 74

accumulation of a variety of polymers in lysosome and ↑ secretion of lysosomal enzymes for their degradation

lacking N-acetylglucosamine phosphotransferase

means phospho-mannose code doesn't make it onto enzymes, they don't go to lysosome, polymers aren't degraded

Front 75

I-cell disease

Back 75

catalytically active enzyme made, but not sorted to lysosome

Front 76

Fabry's

Back 76

catalytically active enzyme made, but activator prot are defective or missing

Front 77

Topology

Back 77

all about occupying equivalent spaces

{ex. gut ~= outside of body; lumen of ER ~= extracellular}

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Golgi

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a series of stacks

"trans" -- near membrane
"cis" -- near nucleus

Front 79

SKL

Back 79

Ser Lys Leu tripeptide

*Import into perozisomes

Front 80

KDEL

Back 80

Lys Asp Glu Leu

return to ER -- retrieves "lost" ER prot

Front 81

Import to Mitochondria

Back 81

~75 a.a. seq.

Front 82

Diseases of Defective Peroxisomal Targeting

Back 82

Zellweger Syndrome
Neonatal Adrenoleukodystrophy

Front 83

How Maternal IgG gets into Baby's Bloodstream

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Transcytosis

Ab binds Fc receptor, taken into cell via endocytosis (assoc. @ ↓ pH)

endosome crosses cell as part of normal mem. recycling, fuses with opposite side, ↑ pH after fusion causes Ab release

Front 84

Functions of Cytoskeleton

Back 84

1) Structural scaffold determining cell shape and polarity
2) cause tension and locomotion ("ropes and pulleys")
3) org. and drive intracellular traffic ("roads and highways")
4) Basis for mitosis and meiosis

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Major Cytoskeletal Components

Back 85

1) Microtubules

2) Actin Microfilaments

3) Intermediate Filaments

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Hyperstable Polymers

Back 86

ex. Cilia and Flagella -- 9+ 2 arras of MTs (axoneme)

Muscle cells have sarcomeres of F-actin and intestinal epithelia have actin bundles in microvilli

Epithelial cells have IFs (tonofilaments) that insert into desmosome junctions

Front 87

Cytoskeleton polymers

Back 87

polarized
organized around specific structures

equilib between monomers/polymers

Front 88

Immotile Cilia (Kartagener's) Syndrome

Back 88

affects humans and dogs
presents with chronic rhinitis, sinusitis, bronchiectasis, male infertility, and assoc. situs inversus of thoracic and abdominal viscera

diag: ciliary/flagellar dyskinesis (ab. or no mvmt)

*lack of or ab. dynein arms

Front 89

Centrioles

Back 89

9 + 0 triplet array of MTs

present as pairs in centrosomes of all cells

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Basal Bodies

Back 90

name given to centrioles that nucleate MTs in axonemes (cilia, flagella)

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Axoneme

Back 91

9 + 2 array found in cilia, flagella

Front 92

Organelle Transport

Back 92

bidirectional , requires MT motors that recog. polarity

Front 93

kinesins

Back 93

family of small dimer prot

ORTHOGRADE transport (cell body to cell periphery)

ex. exocytosis and mvmt of synaptic transmitter vesicles from neuron along axon to synapse

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dyneins

Back 94

group of large multiprot. complexes powering RETROGRADE transport (from periphery to center)

ex. endocytosis and mvmt of synaptic vesicles to cell body

Front 95

Tauopathies

Back 95

Tau -- MT binding protein

proteins control spacing of axonal cytoskeleton can lead to "plumbing problems" if cytoskeletal prot. accumulate.

Front 96

Heredity Spherocytosis (HS)

Back 96

defect in membrane stabilization

RBC bilayer laminated by cytoskeleton of actin, spectrin, ankyrin, bands 4.1, 4.2

osmotic fragility of RBC --> trapping in spleen, RBC destruction, anemia, jaundice, splenomegaly

Front 97

Actin-Based Motility

Back 97

1. Self Assembly
2. Polarity
3. Cross-linkers, motors, membranes
4. Myosin regulation by Ca++

*Actin MFs stabilize membranes and create forces by sliding filament motor mech. or by rapid F-actin growth

Front 98

Cytokinesis

Back 98

myosin-mediated sliding filaments in dividing cells reorganize their MFs into contractile ring

*blocking actin prevents cytokinesis

Front 99

Listeriosis

Back 99

Listeria monocytogenes -- gram +

causes food poisoning, meningitis, spontaneous abortion

enters via intestinal epithelium, hijack F-actin mech, shoot spike into neighboring cell w/ explosive MF growth

Front 100

Intermediate Filaments

Back 100

1. Lamins -- ubiquitious
2. Keratins -- epithelia & related
3. Neurofilaments - neurons
4. Vimentins - fibroblasts
5. GFAPs - glia
6. Desmin - skeletal, cardiac, and smooth muscle

*type specific enough to diagnose cell type origin

Front 101

Epidermal Blistering

Back 101

defect in IF assembly in skin cells

leads to painful and fatal blistering of skin in infants

Front 102

Taxol

Back 102

freezes MTs, prevents disassembly

Front 103

Basic Actin Structure

Back 103

α, β, γ -- 3 types, don't interact.

↑↑ conserved struc. -- * no known disease from actin mut (almost certainly fatal)

*can interrupt assembly, cause disease (ex. leprosy)

Front 104

IF Structure

Back 104

twisted rope -- ↑ strength

Front 105

Desmosomes

Back 105

like a spot weld, 2 cells attached

hemidesmosome -- cell attaches to matrix

Front 106

IF Major Role

Back 106

Mechanical / Structural Integrity

Front 107

Ca Signalling Diseases

Back 107

Malignant Hyperthermia
Migraine Headaches
Fertility
Cardiac Arrhythmias

Front 108

Cell-Cell surface receptor diseases

Back 108

Cholera
Pertussis
ED

Front 109

Nitric Oxide

Back 109

Angina

Front 110

AIS

Back 110

Androgen Insensitivity Syndrome

Front 111

Steroid Communication Diseases

Back 111

COPD
Hypertension
AIS