quiz 4.2 Flashcards
dietary def in vitC , leading to abnorml ollagen
scurvy
deficient type3 collagen
progressive deterioration of collagens
Ehlers-Danlos
dse of type3 collagen
leads to hematuria, end stage renal disease
Alport syndrome
keratin defects
skin blistering
epidermolysis bullosa
deficiency in copper
leads to defective crosslinking of collagen and elastin by lysyl oxidase
menkes dse
overload ot copper
wilsons dse
structural CT proteins
collagen elastin fibrillin keratin fibroins
3 types of structural tissue
collagen
elastin
reticular
the collagen molecule composed of three lefthanded polypeptide helices coiled around each other to form a righthanded superhelix
tropocollagen
distinctive amino acid conposition of collagen
1/3 glycine
1/4 proline
occurence of glycin ein collage is
every third psoition of the alpha chain
100 AAs of the X and Y in gly-X-Y are
proline and hydroxyproline respectively
hydroxylTion of hydroxyproline partciipates in ____ while hydroxylation serves to form ______
interchain hydrogen bonding
attchments for polysacchs
proline hydroxylase requires what in the conversion of proline to hyp
ascorbic acid
sugars are added to collagen by the successive action of what enzymes
gaalctosyl transferase
glucosyl transferase
the carbohydrate with the collage is for what function
directing fibril assembly
glycin is criticla in collagen bcoz
it is small
what part of the proline repel one another by steric hindrance forcing the chain into a thin stretched out helix
pyrrolidine
lysine oxidase converts the epsilon amino group of lysin or hyl to the aldehyde derivated _____
allysine
allysin react blabla to produce ____ to form stable cross link
schiff base
sse of animLs due to ingestion of a certain seed
lathyrism
most seriousnkind of ehlees danlos
type 4
spontaneous rupture of bld bessels
dystrophic form in epidermolysis bullosa is die to
mutation of COL7A1
due to deletion sof alpha1 gene but a single amino acid change is sufficient to cause certain forms of this dse
osteogensis inperfecta
CR protein responsible for extensivility and elastic recoil
elastin
elastic has an _____ made of approximately 830 akino acid
irregular or random coil confirmation
in contrast to colllagen, elastin has no ____ and vey little ____
hydroxylysine
hydroxyproline
a molecular knot that ties 4 chains of elastin net together
desmonsine
cindensation if 3 allysine residues with the epsi-amino acid of a lysine residue gives a product _____
desmosine
disorder of elastin degardation
alpha1-antitrypsin deficiency
tx for AAT deficiency
weekly IV
what causes the emphysema and cirrhosis in AAT deficiency
neutrophils release elastase
kind of intramolecular cross links in collagen
aldol
intramolecular cross link in elastin
desmosine
mutations in the gene for fibrillin-1 leads to ___
marfan syndrome
characterized by ectopia lentis long leg bons and spiderlike fingers enlarged aorta
marfan syndrome
major proteins of hair fingernails and a major fraction of animal skin
alpha keratin
basic atructural unit og keratin
protofibril
basic structural unit of hair
microfibeil
alpha keratin is hardened by introduction of
disulfide croslinks
how is microfibrilformed
8 protofibrils combine either ciruclar or square arrangement
is alpha keratin mono di or polymer?
dimer
structure ng fibroins
gly every other AA tas either ser or ala in between
improrant glycoprotein fir cell adhesion and migration
fibronectin
extracellular glycoprotein that enables eptihelial cells to attach underlying connective tissue
laminin
laminin is a alrge compelx of ___ polypep chains arracged in the shape of a criss and held together by disulfide bonds
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