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Medical Biochemistry > Ch. 10: Fibrous & Non-fibrous Proteins and Proteoglycans > Flashcards

Ch. 10: Fibrous & Non-fibrous Proteins and Proteoglycans Flashcards

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USMLE® Step 1 flashcards
1
Q

What connective tissue cells synthesize proteins & proteoglycans?

A

Fibroblasts (general connective tissue)

Chondroblasts (cartilage)

Osteoblasts (bone)

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2
Q

Type I collagen: Locations? Disorders?

A

BONE, tendon, skin, dentin, facia, arteries

Osteogenesis imperfecta, Ehlers-Danlos syndrome (EDS)

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3
Q

Type II collagen: Locations? Disorders?

A

CARTILAGE, vitreous humor

Chondrodysplasia: spondyloepiphysial dysplasia, achondrogenesis, Stickler syndrome

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4
Q

Type III collagen: Locations? Disorders?

A

Skin, BLOOD VESSELS, uterus

EDS type IV

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5
Q

Type IV collagen: Locations? Disorders?

A

BASEMENT MEMBRANE

Alport syndrome: nephritis, sensorineural deafness

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6
Q

Type V collagen: Locations? Disorders?

A

Skin, placenta, blood vessels, chorion uterus

EDS types I, II

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7
Q

Ehlers-Danlos syndrome (EDS): 6 types?

A 
Hypermobility
Classical
Vascular
Kyphoscoliosis
Arthrochalasia
Dermatosparaxis
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8
Q

Osteogenesis imperfecta (OI): types due to quantity of collagen? Quality of collagen?

A

Quantity: types I, III, IV

Quality: type II (death)

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9
Q

Scurvy: deficiency in which vitamin? effect on collagen?

A

Vit C

Compromise quality of collagen (problem with hydroxylating Pro & Lys)

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10
Q

Elastin: rich in which a.a.s? Locations?

A

Pro, Lys

Lungs, walls of large arteries, elastic ligaments

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11
Q

Describe elastin structure (2 things)

A

Tropoelastin interact with fibrillin

Desmosine cross links by lysyl oxidase

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12
Q

What is alpha1-antitrypsin?

A

Protease inhibitor (major role: inhibit neutrophil elastase)

Synthesized in liver, monocytes, and macrophages

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13
Q

Fxn of alpha1-antitrypsin in lungs?

A

Inhibits elastase ➡️ prevents alveolar wall damage

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14
Q

What happens in emphysema?

A

Alpha1-antitrypsin deficiency/destruction ➡️ elastase destroys elastin indiscriminately

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15
Q

What mutation causes 2-5% of emphysema? What type of inheritance?

A

E342K (Glu ➡️ Lys / GAG ➡️ AAG at 342)

Autosomal recessive

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16
Q

a.a. problem in smokers with alpha1-antitrypsin deficiency?

A

Inactive Met in alpha1-antitrypsin

17
Q

What causes Marfan syndrome?

A

Defect in fibrillin gene

18
Q

What are proteoglycans (mucopolysaccharides)?

A

Long, linear chains of glycosaminoglycans attached to core protein

19
Q

What are the chains of proteoglycans made of?

A

Repeating disaccharide (➖ charge): hexoseamine + uronic acid

20
Q

Fxn of proteoglycans?

A

Part of connective tissue (participate in organization of ECM)

21
Q

Glycosaminoglycans: Hyaluronate

Composition?

A

D-glucosamine + D-glucuronate

No sulfate linkages

22
Q

Glycosaminoglycans: Chondroitin sulfate

Composition?

A

D-galactosamine + D-glucuronate

23
Q

Glycosaminoglycans: Dermatan sulfate

Composition?

A

D-galactosamine + L-Iduronate or D-glucuronate

24
Q

Glycosaminoglycans: Keratan sulfate

Composition?

A

D-glucosamine + D-galactose (no uronic acid)

25
Q

Glycosaminoglycans: Heparan sulfate

Composition?

A

D-glucosamine + D-glucuronate (major), L-iduronate (minor)

26
Q

Glycosaminoglycans: Heparin

Composition?

A

D-glucosamine + L-iduronate (major), D-glucuronate (minor)

27
Q

What are mucopolysaccharidoses (MPS)?

A

Genetic disorders of proteoglycan metabolism

Excessive accumulation & excretion of glycosaminoglycans

28
Q

What causes mucopolysaccharidoses (MPS)?

A

Deficiencies of lysosomal enzymes (fxn: degrade proteoglycans)

29
Q

MPS I H (Hurler), MPS I S (Scheie), MPS I H/S (Hurler/Scheie):

Enzyme deficiency? Glycosaminoglycan affected?

A

Alpha-L-iduronidase

Dermatan sulfate, heparan sulfate

30
Q

MPS II (Hunter)

Enzyme deficiency? Glycosaminoglycan affected?

A

Iduronate sulfatase

Dermatan sulfate, heparan sulfate

31
Q

Peptidoglycans: Made of? Part of?

A

Heteropolysaccharide chains cross-linked by short peptide chains

Bacteria cell walls; have antigenic determinants

32
Q

Describe Gram ➕ bacteria

A

Cell wall: peptidoglycans & teichoic acids

33
Q

Describe Gram ➖ bacteria

A

Peptidoglycans + outer membrane system external to plasma membrane, NO teichoic acids

Medical Biochemistry (23 decks)

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