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36 Cards in this Set
- Front
- Back
- 3rd side (hint)
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What is the Cellular component of hemidesmosomes?
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α-keratin
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What is the Extracellular component of hemidesmosomes?
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Laminin
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What is the Central component of hemidesmosomes?
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Integrin
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Structure of Integrin
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Heterodimer
18 α-chains; 8 β-chains |
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Adhesion protein in desmosomes
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cadherin
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How does the helical structure of the α-keratin differ from that of DNA
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5.1 angstroms in keratin versus the normal 5.4
This allows for more flexibility. |
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What are the obligatory heterodimer expression pairs for Keratin, and which layers are they found
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K1/K10 (Spinous layer)
K5/K14 (Basal layer) |
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What is the genetic problem in Epidermolysis Bullosa Simplex
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Missense mutation in KRT5 or KRT14
results in defective keratin filaments K5/K14--> blistering at basal layer |
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What is the genetic problem in Epidermolytic hyperkeratosis
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Missense mutation in KRT1(carboxyl end) or KRT10 (Amino end)
results in defective keratin filaments K1/K10--> blistering at suprabasal layer |
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How does hylanuronate get out of the cell
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ABC transporter + ATP
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How does hylanuronate differ from other GAGs
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it is not covalently attached to a protein
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What are the GAG's
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Chondroitin sulfate
Dermatan Sulfate Heparan Sulfate Keratan Sulfate (hylanuronate) |
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Where does proteoglycan synthesis occur
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Golgi
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KRT5 is located on what chromosome
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Chr 12
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KRT14 is located on what chromosome
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Chr 17
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What helps control the synthesis of proteoglycans and GAG's (Mucins) and the keratin levels
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Vitamin A
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What are the two structural proteins
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collagen
Elastin |
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What are the two adhesive proteins
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Fibronectin
Laminin |
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Major Collagen type found in the basal lamina (H)
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Type IV
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Floor
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Most common type of the collagen in the body (90%)
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Type I (Fibrillar)
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What type of collagen helps weld two pieces of fibrin (H)
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Type IX
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Nine Nails them together
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Primary forms of collagen found in skin
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Types I and III
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Collagen uses a Triple helix. It has a repeating triple sequence, and every third A.A. is...
Why? |
Glycine
R grp- is a proton (H+) so it fits |
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What A.A. disrupts the alpha helix structure, creating the stronger collagen structure?
How does it make it stronger |
Proline
Its R-grp folds back on itself. So H-bonding is not possible by the amides, but the carboxyl oxygen can H-bond. |
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Enzyme involved in Scurvy?
What is deficient that causes this? |
Prolyl Hydroxylase
Ascorbic acid |
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Enzyme involved in Ehlers-Danlos VI?
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Lysyl Hydroxylase
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Enzyme affected in Ehlers-danlos V
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Lysyl Oxidase
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Co-factors for Lysyl Oxidase
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Cu2+ and Vit. B6
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Co-factors for Prolyl/Lysyl Hrodroxylases
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Ascorbate
Fe2+ |
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These three A.A.'s in sequence bind cell integrins in the basal lamina.
(Remember this!!!) |
Arg-Gly-Asp
R-G-D |
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Osteogenesis Imperfecta:
Defect in what Collagen type? |
Type I
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Ehlers-Danlos IV:
s/s? Defect in what Collagen type? |
Fragile skin and blood vessels
Collagen type III |
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Ehlers-Danlos VII:
s/s? Defect in what Collagen type? |
Easily bruised & hyperextensible
Collagen type I |
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Ehlers-Danlos V:
s/s? Defect in what enzyme? |
Poor wound healing & hyperextensible
Lysyl Oxidase defect |
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Ehlers-Danlos VI:
s/s? Defect in what enzyme? |
Poor wound healing & hyperextensible
Lysyl hydrolase defect |
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Epidermolysis bullosa:
s/s? Defect in what? |
Blistering skin
Keratin defect |
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