Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Musculoskeleton > Biochemistry Of connective Tissue ECM > Flashcards

Biochemistry Of connective Tissue ECM Flashcards

1
Q

Fibrin-forming types of collagen

A

Type 1-3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Type 1 collagen

A

Forms fibrils in skin,bone,tendon, blood vessels and cornea

  • made up of 2 (a1) and 1 (a2) chains
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Type 2 collagen

A

Fibrin-forming in cartilage, intervertebral disks, vitreous bodies

-made up of 3 (a1) chains

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Type 3 collagen

A

Forms fibrils in blood vessels, skin and muscle

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Network-forming types of collagen

A

Types 4 and 8

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Type 4 collagen

A

Network-forming collagen found in basement membrane

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Type 8 collagen

A

Network-forming collagen found in corneal and vascular endothelium

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Fibril-associated collagen types

A

Types 9 and 12

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Type 9 collagen

A

Fibril-associated collagen found in cartilage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Collagen type 12

A

Fibril-associated collagen found in tendons and Ligaments

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Collagen basic structure

A

Fibrous protein shaped in via 3-(a) chains. Stabilized by intrachained H-bonds.

  • each (a)-chain is encoded by a different gene
  • Rich in Gly and Pro amino acids.
  • every 3rd amino acid is a “Gly”
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Hydroxylated amino acids found in collagen

A

HydroxyPROLINE

hydroxyLYSINE

  • requires, oxygen, iron and vitamin C (ascorbate) to hydroxylate amino acids*
  • occurs in rough ER
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Cross linking of collagen

A

Cooper-containing extracellular enzymes delaminates lysine and hydroxylysine residues to reactive aldehyde groups.

These aldehyde groups can cross link with each other in mature collagen fibers, further promoting tension strength.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Collagen synthesis occurs where?

A

Endoplasmic reticulum = procollagen (immature) formation

Extracellular matrix = mature collagen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Steps of transforming procollagen into collagen in the extracellular matrix

A

N and C proteinases cleave propeptides

  • collagen fibrils are formed
  • Lysyl oxidase begins formation of aldehyde groups that covalently crosslink with each other.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Collagenopathies

A

Genetic diseases due to defects in one of the steps of collagen fiber synthesis

17
Q

Elastic fiber composition

A

Inner core of elastin protein surrounded by microfibils of fibrilin

  • very interconnected via cross linking, allowing it to “bounce back” to its original structure.
  • lysyl oxidase is the enzyme that does the cross-linking.
18
Q

Fibrillins

A

Glycoproteins that provide scaffold for elastin

major group is Fibrillin-1

19
Q

Laminin

A

Large cross shaped glycoproteins that consists of 3 polypeptide chains encoded by different genes (heterotrimer)

Functions to hold components of the basement membrane together, trigger physiological responses, growth and movement of the body.

Quarternary protein

20
Q

Fibronectin

A

large protein formed by two polypeptide chains linked by disulfide bonds. Binds to heparan sulfate proteoglycans.

Functions to glue cells to fibrous matrix in ECM, movement of cells In embryogenesis and metastasis of cancer

Most abundant multi-adhesive protein

21
Q

Proteoglycans vs Glycoproteins

A

Proteoglycans: high carb, low protein percentages
(95% carbs: 5% proteins)
Heteropolysaccharide chains

Glycoproteins: high protein, low carb percentages

22
Q

Proteoglycans functions

A

Gel-like matrix that provides flexible support in ECM and lubricating properties.

Influences movement of material through ECM

Lubricating properties of mucus

23
Q

Glycosaminoglycans (GAGs)

A

Large complexes of negatively charged heteropolysaccharide chains.

Formed by a repeating disaccharide unit of an acidic sugar and an N-acetylated amino sugar.

Attracts water via the N-acetylated sugar

6 major classes Sorted by composition, type of glycosidic linkage and degree/location of sulfate units.

24
Q

How is collagen synthesized

A

Synthesized in the cells of CT in their endoplasmic reticulum.

Ribosomes synthesize the individual chains of collagen fibers.

Move to the lumen of the ER and hydroxylation and glycosylation of the proline/lysine residues
Forms the procollagen

Moves to extracellular matrix via transport vesicles Where the terminals of procollagen are cleaved

Lysyl oxidase then binds procollagen fibers together to generate mature collagen

25
Q

Structures in proteoglycans aggregates

A

GAGs

A core protein

Hyaluronic acid

A link protein

25
Q

Chondroitin 4-6 sulfate

A

Class of GAG

Most abundant GAG in the body that is found in cartilage, tendons ligaments and aorta.

From aggregates via noncovalent bonds with hyaluronic acid

26
Q

Keratan sulfates

A

Class of GAG

Most heterogenous GAG containing additional monosaccharides

Found in cornea (subtype 1) and loose connective tissue (subtype 2).

Aggregates with non-covalent bonding with chondroitin sulfate

27
Q

Hyaluronic acid

A

Class of GAG

NOT sulfates and not covalently bound to any protein.

Found in bacteria as well as animal tissue

Serves as lubricant and shock absorber

Found in synovial fluid of joints, vitreous humor of the eye, loose CT and cartilage.

28
Q

Dermatan sulfate

A

Class of GAG

Found in skin, blood vessels and heart valves

29
Q

Heparin

A

Class of GAG

(A)-sulfur linkages joins disaccharides, only one to do so

ONLY INTRACELLULAR GAG, in Mast cells exclusively

Serves as an anticoagulant

30
Q

Heparan sulfate

A

Class of GAG

Found in basement membrane and as a ubiquitous component of cell surfaces.

31
Q

Metabolism of GAGs

A

Synthesized in this order

  • core protein
  • carb chains
  • sulfonation post-modification in carb chains

Degradation:
By endocytosis and Lysosomes

32
Q

Chondrosytrophies

A

Defects in the sulfonation of GAGs (affects synthesis)

Autosomal recessive

Causes dwarfism

33
Q

Mucopolysaccharidoses

A

Deficiency of lysosomal hydrolysis in heparan sulfate or dermatan sulfate (affects degradation)

Autosomal recessive
-except for Hunter syndrome subtype (X-linked recessive)

Progressive disorder with GAGs accumulating in lysosomes

Oligosaccharides in the urine is present

Causes skeletal and ECM deformities and mental retardation.

34
Q

Hurler syndrome (MPS 1)

A

Subtype of mucopolysaccharidoses

Deficient of L-iduronidase (cant degrade dermatan and heparan sulfate)

Affects corneas of eyes and coronary arteries leading to ischemia. Coronal clouding

Treated by bone marrow transplants and enzyme replacement therapy

Most severe form and one of the most common types

35
Q

Hunter syndrome (MPS 2)

A

Subtype of mucopolysaccharidoses

iduronate sulfatase deficiency (cant degrade dermatan and heparan sulfate)

X-linked recessive

Mild-severe physical deformities (specifically rashes) No coronal clouding.

Treated via enzyme replacement therapy

One of the most common types

36
Q

Sanfilippo Syndrome (MPS 3)

A

Affects various steps of removing sulfate residues from heparan sulfate

Severe nervous system disorders and developmental disorders

No coronal clouding

37
Q

Sly syndrome (MPS 7)

A

Glucuronidase deficiency (impairs degradation of dermatan and heparan sulfate)

Hepatosplenomegaly, Skeletal deformity, short store and corneal clouding

Musculoskeleton (36 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions