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MedBiochem > quiz 4.2 > Flashcards

quiz 4.2 Flashcards

1
Q

dietary def in vitC , leading to abnorml ollagen

A

scurvy

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2
Q

deficient type3 collagen

progressive deterioration of collagens

A

Ehlers-Danlos

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3
Q

dse of type3 collagen

leads to hematuria, end stage renal disease

A

Alport syndrome

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4
Q

keratin defects

skin blistering

A

epidermolysis bullosa

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5
Q

deficiency in copper

leads to defective crosslinking of collagen and elastin by lysyl oxidase

A

menkes dse

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6
Q

overload ot copper

A

wilsons dse

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7
Q

structural CT proteins

A 
collagen
elastin
fibrillin
keratin
fibroins
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8
Q

3 types of structural tissue

A

collagen
elastin
reticular

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9
Q

the collagen molecule composed of three lefthanded polypeptide helices coiled around each other to form a righthanded superhelix

A

tropocollagen

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10
Q

distinctive amino acid conposition of collagen

A

1/3 glycine

1/4 proline

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11
Q

occurence of glycin ein collage is

A

every third psoition of the alpha chain

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12
Q

100 AAs of the X and Y in gly-X-Y are

A

proline and hydroxyproline respectively

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13
Q

hydroxylTion of hydroxyproline partciipates in ____ while hydroxylation serves to form ______

A

interchain hydrogen bonding

attchments for polysacchs

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14
Q

proline hydroxylase requires what in the conversion of proline to hyp

A

ascorbic acid

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15
Q

sugars are added to collagen by the successive action of what enzymes

A

gaalctosyl transferase

glucosyl transferase

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16
Q

the carbohydrate with the collage is for what function

A

directing fibril assembly

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17
Q

glycin is criticla in collagen bcoz

A

it is small

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18
Q

what part of the proline repel one another by steric hindrance forcing the chain into a thin stretched out helix

A

pyrrolidine

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19
Q

lysine oxidase converts the epsilon amino group of lysin or hyl to the aldehyde derivated _____

A

allysine

20
Q

allysin react blabla to produce ____ to form stable cross link

A

schiff base

21
Q

sse of animLs due to ingestion of a certain seed

A

lathyrism

22
Q

most seriousnkind of ehlees danlos

A

type 4

spontaneous rupture of bld bessels

23
Q

dystrophic form in epidermolysis bullosa is die to

A

mutation of COL7A1

24
Q

due to deletion sof alpha1 gene but a single amino acid change is sufficient to cause certain forms of this dse

A

osteogensis inperfecta

25
Q

CR protein responsible for extensivility and elastic recoil

A

elastin

26
Q

elastic has an _____ made of approximately 830 akino acid

A

irregular or random coil confirmation

27
Q

in contrast to colllagen, elastin has no ____ and vey little ____

A

hydroxylysine

hydroxyproline

28
Q

a molecular knot that ties 4 chains of elastin net together

A

desmonsine

29
Q

cindensation if 3 allysine residues with the epsi-amino acid of a lysine residue gives a product _____

A

desmosine

30
Q

disorder of elastin degardation

A

alpha1-antitrypsin deficiency

31
Q

tx for AAT deficiency

A

weekly IV

32
Q

what causes the emphysema and cirrhosis in AAT deficiency

A

neutrophils release elastase

33
Q

kind of intramolecular cross links in collagen

A

aldol

34
Q

intramolecular cross link in elastin

A

desmosine

35
Q

mutations in the gene for fibrillin-1 leads to ___

A

marfan syndrome

36
Q

characterized by ectopia lentis long leg bons and spiderlike fingers enlarged aorta

A

marfan syndrome

37
Q

major proteins of hair fingernails and a major fraction of animal skin

A

alpha keratin

38
Q

basic atructural unit og keratin

A

protofibril

39
Q

basic structural unit of hair

A

microfibeil

40
Q

alpha keratin is hardened by introduction of

A

disulfide croslinks

41
Q

how is microfibrilformed

A

8 protofibrils combine either ciruclar or square arrangement

42
Q

is alpha keratin mono di or polymer?

A

dimer

43
Q

structure ng fibroins

A

gly every other AA tas either ser or ala in between

44
Q

improrant glycoprotein fir cell adhesion and migration

A

fibronectin

45
Q

extracellular glycoprotein that enables eptihelial cells to attach underlying connective tissue

A

laminin

46
Q

laminin is a alrge compelx of ___ polypep chains arracged in the shape of a criss and held together by disulfide bonds

A

3

MedBiochem (16 decks)

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