L10 Flashcards

1
Q

the connective tissue or extracellular matrix refers to the complex material surrounding the cell tissue and it includes?

A

collagen

elastin

laminin

fibronectin

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2
Q

what is the most abundant protein in mammals ?

A

collagen , predominantly in connective tissue

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3
Q

what are the functions of collagen ?

A

gives strength and support to the tissue

contributes to proper allignment of cells . which in turn helps in cell proliferation and differentiating into different tissues

collagen in blood vessels also contributes to blood clotting ( thrombus formation )

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4
Q

collagen is not a single protein , rather is it a group. how many types are there ?

A

19 different types.

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5
Q

where can collagen type 1 and 2 be found ?

A

in skin and bone

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6
Q

describe the structure of collagen ,

A

triple helix

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7
Q

what is the basic structural unit of collagen ?

A

tropocollagen

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8
Q

what is each tropocollagen molecule made of ?

A

3 alpha chains , each alpha chain contains a repeated sequence of glycine-X-proline/hydroxyproline

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9
Q

what contributes to 1/3rd of collagens amino acid composition ?

A

glycine

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10
Q

each chain forms a ____

A

left handed helix , three chains join to form a right handed triple helix , which gives collagen its strength

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11
Q

what gives collagen its strength ?

A

covalent cross links formed between lysine and hydroxy lysine residues

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12
Q

the degree of collagen cross linking increases with ——

A

age, thus , in older people , the collagen containing tissues (e.g. skin, blood vessels) become less elastic and stiffer, contributing to health complications.

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13
Q

give some examples of inherited diseases of collagen synthesis

A

Ehlers-Danlos syndrome

Osteogenesis imprefecta

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14
Q

give an example of acquired disease of collagen synthesis .

A

This is a disease due to the deficiency of vitamin C (ascorbic acid).
Vitamin C is needed to hydroxylate lysine and proline to form the cross links in collagen.
Vitamin C deficiency results in unstable and weak collagen.
Scurvy is characterized by bleeding of gums, poor wound healing and subcutaneous hemorrhages.

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15
Q

describe Ehlers-Danlos syndrome

A

comprises a group of inherited disorders
principal clinical features are hyper-extensibility of the skin
abnormal tissue fragility and increased joint mobility

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16
Q

describe Ostepgenesis imprefecta

A

Extremely fragile bones
Caused by a defect in the gene that produces type 1 collagen, an important building block of bone.
The severity depends on the specific gene defect.

17
Q

what is elastin ?

A

it is an important connective tissue protein that is mainly responsible for the extensibility and elasticity of tissue

18
Q

where can elastin be found in large quantities ?

A

Elastin is found in large quantities in Lungs, arterial blood vessels, elastic ligaments etc.

19
Q

how is elastin synthesized ?

A

as tropo-elastin

20
Q

what are some abnormalities associated with elastin production ?

A

Williams syndrome is a genetic disease due to impairment in elastin synthesis.
Decreased synthesis of elastin is found in aging of skin and pulmonary emphysema

21
Q

what is fibronectin ?

A

cell surface protein that is closley involved in the interaction of cells with extra cellular matrix

it actively participates in cell adhesion and cell migration

22
Q

what is laminin ?

A

basal lamina of glomerular membrane ( of renal cells ) contain laminin

23
Q

what are keratins ?

A

structural fibrous proteins found in hair , skin , nails , horns , etc

polypeptide of keratinn form alpha helical structure and are held together by disulfide bond . the toughness and strength of keratin is directly related to disulfide bonds