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biochemistry > proteins > Flashcards

proteins Flashcards

1
Q

what is connective tissue

A

complex extracellular matrix that fills the space between cell and binds cells and tissue together

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2
Q

what are the functions of connective tissue

A

provides support and connecting frame work for all other tissue

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3
Q

name the three major classes of biomolecules in extracellular matrix

A
  1. the structural proteins: collagen, elastin, & fibrillin
  2. specialized protein: fibronectin & laminin
  3. proteoglycans
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4
Q

what are the functions of ECM

A
  1. protection: protect brain from skull
  2. structural support: bone
  3. connection & binding: ligament, tendon
  4. storage: bone stores ca & p
  5. transportation: blood
  6. immune function: WBC
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5
Q

name the elastic protein found in tissue where stretch is needed

A

elastin

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6
Q

elastin is a ___________ polypeptide that contain _________ fiber as major component
a) beta plates, , collagen
b) alpha chain, fibronectin
c) linear, elastic

A

c) linear, elastic

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7
Q

what are the properties of elastin

A

stretch and resume their original length
high tensile structure

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8
Q

where can we find elastin

A

Elastic ligaments, walls of arteries, lungs and vocal cord, epiglottis, ligamenta flava
Smaller quantities found in skin, ear cartilage etc.

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9
Q

elastin is rich in ______________amino acids

A

nonhydroxylated proline, lysine, glycine

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10
Q

unlike collagen elastin can exist on _______________ forms

A

no glycosylated

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11
Q

tropoelastin with fibrillin forms

A

scaffolding

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12
Q

cross linking occurs extracellularly via

A

lysyl oxidase

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13
Q

lysyl oxidase gives elastin what property

A

elastic property

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14
Q

describe the structure of elastin

A

highly cross linked insoluble, amorphous structure

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15
Q

true or false
hydroxyproline (1%) present, but no hydroxylysine.

A

true

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16
Q

which protein bind to tropoelastin to form elastic fiber

A

fibrillin

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17
Q

name the enzymes that can degrade elastin protein

A

elastase enzyme such as neutrophil-elastase

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18
Q

what inhibits a no of protease including neutrophil-elastase

A

α1- antitrypsin

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19
Q

what is α1- antitrypsin

A

is a class of protease inhibitors that normally inhibits elastase

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20
Q

α1-antitrypsin is synthesized by

A

liver

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21
Q

α1-Antitrypsin deficiency results in

A

unopposed elastase activity, which can cause COPD

22
Q

PANACINAR Emphysema caused by

A

deficiency of α1- antitrypsin.

23
Q

deficiency of α1- antitrypsin. can cause

A

loss of inhibitory action on neutrophil elastase
excessive destruction of elastin in lung alveolus
excess elastase activity

24
Q

name the disease
-severe in the lower lobes
-associated with 𝜶1-antitrypsin deficiency but can also be found in patients in whom no genetic abnormality has been identified.

A

panacinar emphysema

25
Q

which type of emphysema is usually predominates in the upper lobes

A

centriacinar emphysema

26
Q

glycoprotein that cell adhesion & migrate

A

fibronectin

27
Q

insoluble monomers involved in linking in

A

cell surface integrins to ECM

28
Q

state two characteristics of fibronectin

A

soluble protein
assists in blood clotting

29
Q

what is sequence of aa that binds to the receptor of fibronectin

A

Arg-Gly-Asp

30
Q

what are the functions of fibronectin

A

-binding heparin fibrilin and collagen
-cell migration
-cell morphology
-embryogenesis
-cytoskeletal organization
-attach cells to various components of the ECM
-can circulate in plasma to assist with clotting
-biofilm formation via cell adhesion to basement membrane

31
Q

name the abnormality that cause early onset of scoliosis and skeletal abnormalities

A

spondylometaphyseal dysplasia

32
Q

spondylometaphyseal dysplasia is associated with

A

mutation in fibronectin

33
Q

which protein is the only one that exists as an insoluble dimer as well as a soluble clotting factor

A

fibronectin

34
Q

which is the major protein component of renal glomerular and other basal laminas

A

laminin

35
Q

how many elongated polypeptide chains in laminin

A

3
α, β1, β 2

36
Q

shape of laminin protein

A

cruciform shape

37
Q

what are the binding sites of laminin

A

type 4 collagen, heparin & integrins on cell surface and entactin (nidogen)

38
Q

what is fibrillin

A

a glycoprotein found in many tissue

39
Q

fibrillin is secreted into ____________ by _________

A

ECM, Fibroblast

40
Q

which protein provides a scaffold for deposition of elastin

A

fibrillin

41
Q

name the disease caused by mutation in the gene coding for fibrillin on chromosome 15

A

marfans disease

42
Q

Marfans disease is inherited as an ____________ trait

A

autosomal dominant trait

43
Q

name the disease that affect eyes and cause ectopia lentis

A

marfans disease

44
Q

name the disease has following presentations
long extremities including fingers
scoliosis
myopia and lens dislocation (upward dislocation)

A

marfans disease

45
Q

what is scoliosis

A

sideways curvature of the spine (often diagnosed in adolescent)

46
Q

how marfan’s syndrome affect skeletal system

A

hyper extensibility of joints, tall sature

47
Q

how marfan’s syndrome affect CVS

A

dilatation of aorta( aortic aneurysm and dissection)

48
Q

deficiency of which enzyme cause homocystinuria

A

cystathionine synthase deficiency

49
Q

symptomsw

A
50
Q

difference

A
51
Q

what is proteoglycans

A

they are protein that contain covalently linked glycosaminoglycans
major component of ECM

52
Q

proteoglycans are formed by polysaccharide called

A

glycosaminoglycans( GAH, 95%), protein (1%)

biochemistry (12 decks)

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