Foundations Of Medicine: Cellular Matrix/ecm

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binding sites on beta subunit of laminin

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heparan sulfate, proteoglycan, integrin

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binding sites on gamma subunit of laminin

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entactin, proteoglycan, integrin

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lamina densa contains

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type IV collagen

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entactin

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has a collagen binding site of lamina densa and a laminin binding domain

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integrins

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allow interactions between cells and basal lamina

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junctional epidermolysis bullosa

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epithelial tissue is separated from basement membrane

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fibronectin

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binds collagen/binds cell- dimer by disulfide bonds

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proteoglycan

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contains GAG side-chains
bind to laminin network
can sequester growth factors

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collagen

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has glycine every third and is rich in proline and hydroxyproline
alpha helix

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procollagen

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registered peptides that allow chains to assemble- RER

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procollagen peptidases

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cleave non-helical portions = tropocollagen

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tropocollagen aggregates to form

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fibrils

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fibrils assemble to form

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collagen fibers

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elastic fibers

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tropoelastin
fibrilln I
fibrillin II
microfibril associated glycoproteins
assembled in extracellular environment

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Marfan Syndrome

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deficiency in fibrillin I
tall, long arms, legs, fingers, toes
mitral valve prolapse
dilation of root of aorta
aortic dissection

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matrix metalloproteinases

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depend on metal ions
can degrade ECM proteins

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6 ways MMPs can be regulated

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protelytic activation
proteolytic processing/inactivation
protein inhibiton (TIMP)
ECM localization
cell surface localization
endocytes and intracellular degredation

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Mast cells

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have IgE receptors

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when antigen binds mast cells:

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Ca2+ is mobilized and this causes the release of granules which store histamines, proteases, and proteglycans
cytokines are also released

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eosinophil

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combat parasitic infections

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eosinophil properties

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crystalline center

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____and ____ can both be moved in the cell

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MF and MT

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MT brought to focal adhesion by_____or_____. the contact causes__________ and______.

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elongation or motors
GTP cap destabilized
MT retracts and breaks focal adhesion

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contraction of smooth muscle regulated by

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phosphorylation of myosin light chains

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Lamina Densa is made up of

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type IV collagen

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Type IV collagen has __ domains. These are:

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3
non-collagenous domain 7S
collagenous doman (triple helix)
Non-collagenous domain NC1

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entactin

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binds laminin and collagen IV together

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lamina reticularis

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connects the basal lamina to connective tissue/stroma
made up of collagen III

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Fibronectin

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allows connective tissue cells to interact with basement membranes

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fibrinectin structure

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collagen binding domain
cell binding domain (through integrins)
dimer formed by disulfide bonds at the C-term end

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proteoglycan

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ECM protein
binds laminin network

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structure of proteoglycan and what this can do

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glycosaminoglycan side chains(contains heparan sulfate or chondroitin sulfate)
these sequester growth factors

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BM is connected to ECM by___

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fibromectin and proteoglycan and their interactions with integrins located on the cell membrane

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BM is connected to the cytoskeleton by__________

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binding integrins which are transmembrane proteins and these in turn bind to attachment proteins(talin, vinculin, and alpha actinin) that attach to actin

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cytokinesocytes

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generate signaling complexes that effect distributions of tight junction components

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desmosomes

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tensile strength

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pemphigus foliaceus

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autoantibody
desmoglein 1 antibodies
loss of adhesion of keratinocytes in epidermis

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gap junctions

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no connection of cytoplasm
6 connexins make connexon
regulated by cAMP
cell specific

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hemidesmosomes

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connect IFs of cells to proteins in basal lamina

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hemidesmosomes bind ____ protein in membrane

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integrin

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adapter proteins of hemidesmosomes

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plectin/ BP230

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bullous pemphigoid

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caused by autoantibodies of BP230

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two other proteins that aid in hemidesmosomes

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collagen VII and laminin V

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epidermolysis bullosa

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mutation in laminin 5 which helps connect the epidermis to the dermis

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focal adhesions

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connect actin to extracellular proteins in basal lamina
uses integrins

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adapter proteins

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alpha-actinin, talin, filamin, vinculin

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selectins are found on

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endothelial cells which secrete the selectin when stiumulated by cytokines

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ICAMS

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secreted by endothelial cells and bind integrin on the leukocytes to stop them
-bind alpha-actinin in cytoplasm and can also bind each other

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binding sites on alpha subunit of laminin

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heparan sulfate

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epidermolysis bullosa simplex

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defect in keratin K5 or K14
severe blistering
early onset

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charcot-marie-tooth disorder

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abnormal neurofilaments
symptoms similar to lateral sclerosis(degredation of motor nerves)

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desmin-related myopathy

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chronic neuromuscular disorder
abnormal neurofilaments

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dilated cardiomyopathy

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mutated desmins(intermediate filaments in muscles)

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Emergy-Dreyfus muscular dystrophy

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mutated lamin A or emerin
limb/girdle muscular dystrophy

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pemphigus foliaceus

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autoantibodies to desmoglein I(cadherin family proteins)
crusty sores
no oral problems

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pemphigus vulgaris

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antibodies to desmoglein 3
blistering on oral cavity or body
intra-epithelial clefting

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bullous pemphigoid

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autoantibodies to hemidesmosome proteins(BP180, BP230, laminin 5, beta4 integrin)

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muscular dystrophies

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mutations of dystrophan

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polycystic kidney disease/retinal degeneration

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defects in kinesis complexes that carry cargo to top of cilia and flagella

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selectin

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binds carbs
weaker than cadherins
slow down leukocytes for extravasation
binds PSGL-1 on leukocytes

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ICAMS

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promote binding between endothelial cells and leukocytes
located on endothelia
binds integrins
bound to alpha actinin in cytoplasm

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integrins

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activated by selectin binding
bind ICAMS

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The basement membrane is found____

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on the basal of epithelium and endothelium
surround all muscle cells and nerve cells

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lamina reticularis is made by

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fibroblasts in underlying connective tissue

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basal lamina contains____ and is made by_____

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lamina rara and lamina densa
epithelial cells

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laminin is composed of

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three polypeptides- alpha, beta and gamma

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the alpha subunit of laminin has________binding domain

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heparan sulfate

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binding sites on gamma subunit of laminin

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integrin, entactin, proteoglycan

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binding sites on beta subunit of laminin

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proteoglycan, heparan sulfate, and integrin

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_______makes up lamina densa

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Collagen Type IV

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Three domains in collagin IV

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non-collagenous domain 7S
collagenous domain (triple helix)
non-collagneous domain NC1

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entactin

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binds the laminin and collagen together

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what binds the basal lamina to the epithelial cells?

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integrin- transmembrane in the cells
This allows cells to move and stay firmly attached

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mutations in integrins or laminin chains result in

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detachment of epithelium from basement membrane

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Integrin function

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transmembrane proteins that are receptors for extracellular matrix proteins(fibronectin and laminin)

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Structure of Integrin

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two subunits, alpha and beta. with many different combinations

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_____is an integrin specific to hemidesmosomes and binds ______

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alpha6beta4
extracellular laminin

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fibronectin

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extracellular matrix protein that binds integrins
it also binds other proteins such as collagen

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laminin

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extracellular matrix protein that binds integrins
it also binds other proteins such as collagen

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in hemidesmosomes, __________binds adapter proteins which in turn bind to __________

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intermediate filaments

integrins(transmembrane)

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plectin

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a protein in the plaque proteins (adapter proteins) that help bind integrins

also cross links IF to other IFs and IFs to MT and MF

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mutations in plectin cause

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epidermolysis bullosa

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BP230

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plaque protein
autoantibodies cause bullous pemphigoid

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Collagen XVII

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transmembrane component of hemidesmosomes
also called BP180- autoantibodies cause bullous pemphigoid

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Collagen VII

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helps connect the dermis to the epidermis
laminin 5 also does this

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laminin 5

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helps connect the dermis to the epidermis

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mutations in collagen VII cause

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epidermolysis bullosa

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focal adhesions connect_______to _______by using______.

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actin cytoskeleton
extracellular proteins
integrins

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vinculin

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actin binding protein
defining feature of focal adhesions