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Molecular Biology of the Cell > Extracellular matrix > Flashcards

Extracellular matrix Flashcards

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1
Q

What is the extracellular matrix?

A

Network of macromolecules filling spaces between cells. Made of fibrillar and non-fibrillar componants

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2
Q

What collagens are found in connective tissues?

A

Type I (bone), II (cartilage) III (blood vessels) all fibrillar

Type IV (basement membrane , non-fibrillar)

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3
Q

What multi-adhesive glycoproteins are found in connective tissue?

A

Fibronectin, Fibrinogen

Laminins (basement membrane)

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4
Q

Which Proteoglycans are found in connective tissue?

A

Aggrecan
Versican
Decorin
Perlecan (basement membrane)

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5
Q

What does the mutated gene affect to cause Osteogenesis imperfecta?

A

Type I collagen

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6
Q

What does the mutated gene affect to cause Marfan’s Syndrome?

A

Fibrillin 1

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7
Q

What does the mutated gene affect to cause Alport’s syndrome?

A

Type IV collagen alpha 5

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8
Q

What does the mutated gene affect to cause Epidermolysis Bullosa?

A

Laminin 5, in all 3 chains

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9
Q

What does the mutated gene affect to cause Congenital Muscular Dystrophy?

A

Laminin 2 Alpha 2 chain

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10
Q

What is an example of disorder of ECM catabolism?

A

mucopolysaccarides / GAGs inability to be degraded

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11
Q

What is Hurler’s Syndrome?

A

Mutation which leads to a loss of enzyme L-Alpha-iduronidase.

results in the buildup of large sugar molecules called glycosaminoglycans (AKA GAGs, or mucopolysaccharides) in lysosomes

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12
Q

What category of disorders can be caused by excess ECM deposition? Give 3 examples

A

Fibrotic disorders

Liver fibrosis - Cirrhosis

Kidney Fibrosis - diabetic nephropathy

Lung fibrosis - iodiopathic pulmonary fibrosis (IPF)

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13
Q

What disorder is Osteoarthiritis an example of?

A

Disorders due to excess loss of ECM where it is needed for support

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14
Q

Why is there a wide variety of connective tissues?

A

Different types of collagen with various arrangement coupled with or without different ECM components can create diversity

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15
Q

What are the characteristics of Diabetic nephropathy? Why does it lead to renal failure?

A

There is an accumulation of extracellular matrix making thick basement membrane, This restricts renal filtration , so can lead to renal failure

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16
Q

What are the characteristics of Alport syndrome?

A

Mutations in collagen IV resultl in abnormally split GBM which is associated with loss of kidney function and hearing loss.

17
Q

What do elastic fibres consist of?

A

Protein elastin core and microfibrils which are rich in protein fibrillin surrounding.

18
Q

What disorder is caused by mutations in fibrillin 1 protein?

A

Marfan’s syndrome

skeletal, ocular and cardiovascular system can be affected. Px can be predisposed to aortic ruptures. Arachnodactyly

19
Q

In the protein Elastin, what are the two segments that alternate along the polypeptide chain?

A

Hydrophobic regions and alpha helical regions rich in alanine and lysine. many of the lysine side chains are covalently cross linked

20
Q

What makes ECM proteins multi-adhesive?

A

Their modular architecture. ie, They are made of protein domains of 50-200 amino acids. Makes them multifunctional and can bind to various components /receptors

21
Q

What are the structural characteristics of laminins?

A

Heterortrimeric proteins made of alpha, beta, gamma chains. Form cross shaped molecules. Large proteins

Each chain molecular weight 160-400kDa

22
Q

How do Laminins act as multi-adhesive proteins?

A

can interact with receptors Integrins and Dystroglycan.
Can self-associate with basement membrane matrix as well as other matrix components : type IV collagen, Nidogen, proteoglycan

23
Q

Diseases such as muscular dystrophy, epidermolysis bullosa, congenital muscular dystrophy arise from what?

A

Absence of alpha 2 chain in laminin 2. Symptoms include hypotonia, weakness and deformities of the joints

24
Q

Extracellular matrix extra info?

A
  • key functions : physical support, mechanical and physiochemical properties of the tissue, influences the growth, adhesion and differentiation of cells and tissues it interacts with, essential for development tissue function and organgenesis

cells found in connective tissues : fibroblast, macrophage, basal lamina, collagen fibre, mast cell, elastic fibre, capillary, glycosaminoglycans, proteoglycans, glycoproteins

  • properties of connective tissues ( tendon and skin = tough and flexible ) ( bone - hard and dense ) ( cartilage = resilient and shock absorbing )

Molecular Biology of the Cell (16 decks)

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