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89 Cards in this Set
- Front
- Back
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 |