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MCB I > ECM > Flashcards

ECM Flashcards

1
Q

Collagen characteristics

A

TRIPLE HELIX of ALPHA-chains

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2
Q

Collagen fn

A

Provides TENSILE STRENGTH

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3
Q

What is collagen made by?

A

Fibroblast

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4
Q

What AA is in a collagen?

A

GLYCINE (@ every 3rd AA!!!)
Proline (& hydroxyproline)
Lysine (& hydroxylysine)

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5
Q

What does proline and hydroxyproline offer to the collage?

A

KINKS

Helps helix formation

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6
Q

Collagen synthesis brief steps

A

1) Pre-procollagen → procollagen after ER sig seq trimmed in rER
2) HYDROXYLATION
3) GLYCOLSYLATION
4) DISULFID formation → aligns & initiates TRIPLE HELIX FORMATION (zipper)

5) secreted and both terminus trimmed → now it is COLLAGEN FIBRIL

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7
Q

What is the purpose of procollagen?

A

prevents premature fibril formation

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8
Q

What is the purpose of hydroxylation in collagen

A

↑ stability

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9
Q

Lack of vit c / asocrbic acid =

A

Less hydroxylation =

↓ stability of TRIPLE HELIX

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10
Q

↓ vit c = ____

A

No hydroxylation

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11
Q

What needs to be done to go from procollagen → collagen?

A

PEPTIDASE trim N & C propeptides

Then SELF ASSEMBLY into collagen FIBRILS

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12
Q

What bond holds together procollagen triple helix?

A

H bonds

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13
Q

What does lysyl oxidase do?

A

DEAMINATES lysines & hydroxylysines

Deamination → makes aldehydes

Needed 4 cross linking

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14
Q

Fibrillar collagen

A

Types 1, 2, 3, 5

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15
Q

FACIT collagen

A

Type 6, 9 & 12

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16
Q

Sheet/network forming collagen

A 
Type 4 (BASAL LAMINA)
And 10
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17
Q

Anchoring fibril =

A

Type 7 collagen

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18
Q

Ehrers danlos syndrome defect

A

Defect in fibrillar collagen synth

Leads to having only elastin

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19
Q

Fn of FACITs

A

Organize fibrils in the ECM

Bid 2 surface of fibrillar collagen

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20
Q

Fn of MMPs

A

Break up ECM collagen (4 diapedesis) aka collagenases

ZiNC dependent E’s

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21
Q

What do MMPs need to fn?

A

ZINC

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22
Q

2 components of ECM

A

Fibrous P’s (collagen & elastin)

& Ground Substance (GAGs, Proteoglycans, laminin, fibronectin…)

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23
Q

What is abundant in elastin?

A

Rich in PROLINE & GLYCINE

But NOT glycosylated & NO HYDROXYLYSINE

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24
Q

What is a fibrillin gene mutation?

A

MARFANS

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25
Q

What binds to elastin and what is its role?

A

FIBRILLIN

Fn = essential 4 ASSEMBLY & integrity of elastic fibers

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26
Q

Marfans

A

Fibrillin mutation

Aortic root

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27
Q

COPD

A

↓ alpha-1 AT

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28
Q

What does smoke do to effect COPD

A

Smoke = INHIBITOR OF a1-AT

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29
Q

What do GAGs do?

A

Covalently link to core protein (except HYLAURONAN) to form PROTEOGLYCAN?

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30
Q

What is unique about hyaluronan

A

Does NOT bind to core protein

Rpting seq of Identical NONSULFATED disacch’s

Made in WOUND HEALING
Joint LUBRICANT
BACKBONE for large proteoglycan complexes

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31
Q

HYALURONAN

A 
NOT CORE PROTEIN BOUND
WOUND HEALING
JNT LUBE
NONSULFATED
BACKBONE
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32
Q

LARGE proteoglycan complex with hyaluronan = ___

A

Aggrecan

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33
Q

Hyaluronic Acid (IS/IS NOT) COVALENTLY bound to proteins

A

IS NOT

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34
Q

4 parts of ECM =

A

Type 4 collagen
Laminin
Entactin/nidogen
Hspg (perlecan)

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35
Q

What type of collagen can be found in the basal lamina

A

4

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36
Q

Laminin role

A

Forms SCAFFOLD with collagen type 4

ORGANIZES assembly of basal lamina by binding to integrins and dystroglycans

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37
Q

What is the role of PERLECAN & SYNDECAN?

A

Bind & RECRUIT GFs

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38
Q

Hspg perlecan

A

Rich in basal lamina of KIDNEY GLOMERULUS

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39
Q

Mmp

A

Zinc

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40
Q

Vit c

A

Hydroxylation

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41
Q

Interstitial matrix vs basal lamina

A

Interstitial matrix = SPACE btw epi & endo thelium

BL = UNDER EPITHELIUM

42
Q

General role of FIBRONECTIN

A

Connects cells to all ECMs

Except type 4 – BL

43
Q

Fibronectin connects to what? At what part of the fibronectin?

A

Fibronectin BINDS 2 INTEGRIN @ RDG SEQ of the fibronectin

44
Q

Fns of fibronectin

A

STABILIZE CELL ADHESION TO ECM
Cell migration
WOUND HEALING

45
Q

2 things that bind to integrins

A

Fibronectin & laminin

46
Q

Integrin

A

Principal cell R’s for binding ECM

47
Q

2 types of integrin anchoring jxns

A 
Focal adhesions (motility)
Hemidesmosomes (strength)
48
Q

Hemidesmosomes PARTS

A

IF (keratin) - PLECTIN - INTEGRIN - LAMININ - COLLAGEN 7

49
Q

FOCAL ADHESION PARTS

A

ACTIN - VINCULIN&TALIN - INTEGRIN - FIBRONECTIN

50
Q

FOCAL ADHESION

A

MOTILITY

51
Q

FAK

A

FOCAL ADHESION KINASE

Integrins signal thru FAK 2 regulate Rho GTPase

52
Q

Bullous pemphigoin

A

HEMIdesmosome

53
Q 
Focal adhesion (fn)
PARTS
A 
Fn = motility
F-ACTIN
VINCULIN&TALIN
INTEGRIN
FIBRONECTIN
54
Q

HEMIDESMOSOME (fn)

PARTS

A 
FN = STABILITY
IFs (KERATIN)
PLECTIN
INTEGRIN
LAMININ
COLLAGEN 7
55
Q

PLECTIN

A

HEMIDESMOSOME ADAPTER P

56
Q

VINCULIN & TALIN

A

FOCAL ADHESION ADAPTER P

Mn: 2 words = 2 adaper P’s

57
Q

G proteins 4 cell motility

A

RAC (lamellipodia)

Cdc42 (filopodia)

58
Q

Cdc42

A

Filopodia (sensor) g protein

59
Q

Rac

A

Lamellipodia (leading edge) g protein

60
Q

Filopodia fn

A

SENSOR 4 cell motility

61
Q

LAMELLIPODIA FN

A

LEADING EDGE (CELL MOTILITY)

62
Q

RAC FN

A

REARRANGES. Actin to STRETCH OUT FRONT IN LAMELLIPODIUM

63
Q

Rho
Rac
Cdc42

A

Rho = STRESS FIBERS & FOCAL CONTACTS

Rac = lamellipodia (leading edge)
CDC42 = filopodia (Sensor)
64
Q

Order of jxns from top (apical) to bottom

A

Tight
Adherens
Desmosomes
Gap

Hemidesmosomes & focal adhesions

65
Q

Adherens

A

Actin filament

66
Q

Desmosones

A

IFs

67
Q

Tight jxn adhesion proteins

A

CLAUDIN

OCCLUDIN

68
Q

TIGHT jxn fn

A

Prevent membrane P’s from slipping down / out of place

Determine cell polarity (apical side)

Allows diffusion of substances between cells (NOT FROM 1 cell to another)

69
Q

Chron’s and ulcerative colitis = issue with what?

A

TIGHT jxns (↓ occludin)

70
Q

Adherens jxns

A

ACTIN - CATENIN - CADHERIN - CATENIN - ACTIN

71
Q

Where will you find CATENIN and what does it do?

A

in ADHERENS JXNS

Bind btw actin & cadherin

72
Q

Cadherin dependent on ___

A

Ca++

73
Q

Cadherin

A

Calcium

74
Q

Demosomes parts

A

IF - DESMOPLAKEN - PLAKOGLOBIN - CADHERIN - PLAKOGLOBIN - DESMOPLAKEN - IF

75
Q

DESMOSOME:
anchor _____
Anchor P’s = _______

A

Anchors IFs

Anchor P’s = DESMOPLAKIN AND PLAKOGLOBIN

76
Q

PEMPHIGUS

A

DESMOSOME CADHERIN DFCT

77
Q

GAP JXN PARTS

A

6 CONNEXINS = 1 CONNEXON

2 CONNEXONS = 1 CHANNEL

78
Q

GAP jxn fn

A

All cell to cell communication. Allow muscles to coordinate response and faster response.

Mediate ELECTRICAL AND CHEMICAL COUPLING

79
Q

GAP JXNS ALLOW FREE PASSAGE OF —

A

IONS, SUGARS, AA, NUCLEOTIDES, 2ND MESSENGERS (cAMP…)

80
Q

Electrical coupling fn of gap jxns

A

RAPID SPREAD OF APs

81
Q

Chemical coupling fn of gap jxns

A

Allow 2nd messengers to flow from cell to cell

82
Q

X -linnked charcot-marie tooth mutation

A

Connexin 32

83
Q

Progressive degeneration of peripheral nerves

Impaired deep tendon reflexes

What is the mutation?

A

Connexin 32

Diz = charcot-marie-tooth neuropathy

84
Q

Leukocyte rolling process

A

1) SELECTIN - R bind = WEAK so leukocyte ROLLS along endothelial cells
2) rolling ACTIVATES INTEGRINS ON LEUKOCYTES
3) integrins bind to ICAM = STRONG bind
4) start diapedesis

85
Q

Child bruised

Mut and disease

A

PLATELET INTEGRIN COMPLEX ABSENT (glycoprotein IIb-IIIa)

Glanzmann’s thrombasthenia

86
Q

Glanzmann’s thrombasthenia

A

Absense of PLATELET INTEGRIN

87
Q

OSTEOGENESIS IMPERFECTA

A

MUT COLLAGEN I

88
Q

EHLERS-DANLOS

A

MUT COLLAGEN 1, 3, 5

89
Q

ALPORT

A

MUT COLLAGEN 4

RENAL FAILURE because THICKENING

90
Q

GOODPASTURE SYNDROME

A

MUT COLLAGEN 4

RENAL FAIL

91
Q

EPIDERMOLYSIS BULLOS

A

MUT COLLAGEN 7

HEMIDESMOSOME

92
Q

OSTEOGENESIS IMPERFECTA MUT. And keyphrase

A

Mut collagen I

Substitude of INVARIANT GLYCINE = severe phenottype

BLUE SCLERA

OI TYPE 2 = FATAL

93
Q

WHICH TYPE. Of ehlers danlos = vascular dfcts and it is a mut in WHICH TYPE of collagen?

A

Type 4 = collagen 3 mut

94
Q

Alport mutation

A

Mut ALPHA 5 CHAIN OF type 4 collagen

Nephritis & DEAFNESS

95
Q

Nephritis and deafness

A

Alport

A5 of t4 coll

96
Q

Alport

A

Mutation

97
Q

Goodpasture

A

Auto-immune

98
Q

HSPG PERLECAN IS RICH IN WHICH REGION?

A

BASAL LAMINA

99
Q

GAGs have what relation and function with relation to the KIDNEY?

A

GAGs serve as a SIZE-SELECTIVE and CHARGE SELECTIVE BARRIER

100
Q

Hyperglycemia does what to HSPG?

A

↓ reg hspg → collagen glycosylation and cross linking.

Glomerular basal mem THICKENS with ↑ in collagen and ↓ in HSPG

Note: Lots of HSPG perlecan in BL of glomerulus. ↑ BG down reg’s HSPG → causes collagen to glycosylate & cross-link → basal mem thickens = harder and needs ↑ P of blood to filter

MCB I (5 decks)

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