Organización célula#2 b 429

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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