organización célula#2 b 429
TRANSCRIPT
Fig. 4-1, p. 75
1 μm
Atom
Aminoacids
Protein
Ribosomes
Virus
Mitochondrion
0.1 nm
Smallestbacteria
1 nm 10 nm 100 nm 10 μm
Chloroplast
Nucleus
10 m1 m100 mm
Electron microscopeLight microscope
10 mm
Typicalbacteria
Red bloodcells
Epithelialcell
Humanegg
Frog egg
Chickenegg
Somenerve cells
Adulthuman
1 mm100 μm
Measurements1 meter = 1000 millimeters (mm)1 millimeter = 1000 micrometers (μm)1 micrometer = 1000 nanometers (nm)
Human eye
meter = 3.28 feet
Área de superficie/volumen
Difusión
Concentraciones adecuadasde solutos
Bacterias anaeróbicas- 3,800 mill
Cianobacterias- 3,500 mill
Presencia significativa de O2 2,300 mill
Protistas Eucariotas- 2,300 mill
Invertebrados y algas- 2,000 a 1,000 mill
Fig. 4-6, p. 81
Plasmamembrane
0.5 μm
Pili
Storage granule
FlagellumRibosome
Cell wall
CapsuleNucleararea
DNA
Célula Eucariota
1 – 10 µm 10 – 30 µm
Serial Endosymbiosis Hypothesis
Figure 29.2
Figure 29.2 Cleavage and Blastocyst Formation
Figure 3.1
• The cell membrane is a phospholipid bilayer with proteins, lipids and carbohydrates.
Figure 3.3
The Nucleus
Figure 3.13
Fig. 4-11, p. 88(c)
0.25 μm
ER continuouswith outer membraneof nuclear envelope
Outernuclearenvelope Nuclear pore
Inner nuclearenvelope
Nuclearporeproteins
Nucleoplasm
2 μm
Nuclearpore
Chromatin
Nucleolus
Rough ER
Nuclearpores
Nuclearenvelope
(a)
(b)
ProgeriaCausa descubierta en el 2013
Mutación en pre-laminina A que causa un “splicing” incorrectodel m-RNA
Fig. 4-12, p. 90
1 μm
ER lumenMitochondrion
Ribosomes
RoughER
Smooth ER
35 miopatías y varios desordenes neurológicos. Debilidad muscular, ceguera, sordera, ataques nerviosos, degeneración muscular, hipertrofia cardíaca, infertilidad, etc.
Herencia maternal
Mitochondrial encephalomyopathy Hypertrophyc cardiomyopathy (t-RNA genes)
Mitochondrial diseases
Group 1- Progressive external ophthalmoplegias • Kearns-Sayre disease Ophthalmoplegia (eye movement) syndrome • Ocular myopathy • Leber's hereditary optic neuropathy–due to a point mutation
Group 2- Mitochondrial encephalomyopathies • Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes • Myoclonus epilepsy with ragged red fibers • Leigh syndrome
Group 3- Undefined mitochondrial encephalomyopathies, eg congenital lactic acidosis
Group 4- Mitochondrial myopathies • Luft syndrome • Enzyme defects, eg ATPase, cytochrome oxidase
Peroxisomas
PeroxisomasMutaciones en 11 genes diferentes para enzimas que cortan los ácidos grasos de cadenas bien largas(very long chain fatty acids).
Problemas severos neurológicos, visual, hepáticos, demielinización, y muerte infantil temprana.
Zellweger’s disease (“ghost peroxisomes”)
Neonatal adrenoleukodystrophy (vlcfa transporter)
X linked adrenoleukodystrophy (myelin trasporter)
Infantile Refsum disease (genes for peroxins)
En 2009 terapia genética paralos ABCD-1 transporters detuvo neurodegeneración endos pacientes con X-ALD
Figure 3.11
Lisosomas
40 condiciones congénitas lisosomales(lysosomal storage diseases)
I-cell diseaseN-acetilglucosaminelabels target protein
Tay-Sach diseaseBN-acetilhexosaminidasaphosphotransferasaGangloside GM2
Gaucher’s diseaseglucocerebrosidaseglucocerebroside
Fig. 4-20, p. 96
1 μm
Granum(stack ofthylakoids)
StromaInnermembrane
Outermembrane
Intermembranespace
Thylakoidmembrane
Thylakoidlumen
Mitochondria and Chloroplasts
Figure 3.1
The Cytoskeleton
Figure 3.5
Fig. 4-21, p. 97
Microtubule
Plasmamembrane
Microfilament
Intermediatefilament
Fig. 4-28, p. 102
Plasmamembrane
Collagen
Fibronectins
Integrin
Intermediatefilament
Microfilaments
Extracellularmatrix
Cytosol
Herpes virus
Sistema de Endomembranas
*
*
The Endoplasmic Reticulum
Figure 3.8
-Detoxificación de sustancias liposolubles con citocromo P450
(tolerancia a drogas y toxicidad carcinogénica)
• Monooxigenasas•(barbitúricos- fenobarbital y tolerancia)
•Hidroxilasa de aril-hidrocarbono- (cancer ratones y humo cigarrillo)
Retículo Endoplásmico Liso
Metabolismo de lípidos:
•Síntesis de esteroides•(Statins-inhibition chloesterol)
•Síntesis de membranas
Metabolismo de glucógeno(glucosa-6-fosfatasa)
Almacenamiento de Ca++
Retículo Endoplásmico Liso
• Síntesis de proteínas (transporte co-traduccional)
• Doblaje correcto de las proteínas y degradación de las incorrectas.
• Glucosilaciones
Retículo Endoplásmico Rugoso
tau tangles - Alzheimer’s diseas and argyrophilic grain diseaseWhen misfolded this otherwise very soluble protein can form extremely insoluble aggregates that contribute to a number of neurodegenerative diseases.
Retículo Endoplásmico Rugoso
The Golgi Apparatus
Figure 3.9
Functions of the Golgi Apparatus
Figure 3.10
ER retention tags (RXR)=(Arg-X-ARG)
Retrieval tags Golgi to ER(KDEL)=(Lys-Asp-Glu-Leu)
Secretory tags
Secretory tagsLysosome enzymesMannose-6-phosphateMPRs (Receptors)
Figure 22–11
Natural Killer Cell Function
Secreciones constitutivas y secreciones reguladas
Secresiones o exocitosis regulada
Zimógenos
Neurotransmisores
Hormonas
A Kinesin Motor
Fig. 5-20, p. 124
Vesicle approaches plasma membrane,
fuses with it, and
releases its contents outside cell.
0.25 μm
1
2
3
1
2 3
Fig. 5-21, p. 125
Folds of plasma membrane surround particle to be ingested, forming small vacuole around it.
Vacuole then pinches off inside cell.
Lysosomes fuse with vacuole and pour potent hydrolytic enzymes onto ingested material.
Ingested bacteria
Glycogen (storednutrients)
Nucleus
Lysosomes
Lysosomes Lysosome Lysosome
2.5 μm
Bacteria
Vacuole
Largevacuole
1 2 3
Pinocytosis and Phagocytosis
Figure 3.26
Receptor-Mediated Endocytosis
Figure 3.25
Fig. 5-23b, p. 126
(b) This series of TEMs shows the formation of a coated vesicle from a coated pit.
0.25 μm
ClathrinAdaptor protein Dynamin
Fig. 5-23a, p. 126
Coatedpit
(a) Uptake of low-density lipoprotein (LDL) particles, which transport cholesterol in the blood.
Endosome
Endosome
Primary lysosome
Clathrinrecycled
Secondarylysosome
Freecholesterol
Clathrin
Cytosol
Uncoatedvesicle
Plasmamembrane
LDLreceptor
LDLparticle
1
2
3
4
5
Coat protein I y II
Caveolin
Botox proteasa que corta a SNARE,interfiere con la contracción muscular,La exocitosis de vesículaspre-sinápticas depende de SNARE
soluble N-ethylmaleimide-sensitivefactor attachment protein (SNAP)
SNARE- Snap Receptor
Lysosome
40 condiciones congénitas
I-cell diseaseTay-Sach diseaseGaucher’s disease
Metabolismo de superóxidos yPeróxido(dismutasa de superóxido ycatalasa)
Detoxificación de alcoholes
Oxidación de ácidos grasos(oxidación β de ácidos grasos)
Mutaciones 11 genes diferentes
Zellweger’s disease
Neonatal adrenoleukodystrophy
Infantile refsum disease