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Biochem Fall > Chapter 10- Imi Biochem > Flashcards

Chapter 10- Imi Biochem Flashcards

1
Q

Collagen and elastin form

A

connective tissue- protein fibers embedded in a matrix of proteoglycans (ground substances)

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2
Q

Function of collagen

A

Provide support for the organs and other structures of the body

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3
Q

The proteins and proteoglycans are synthesized by connective tissue cells

A

fibroblasts (generalized connective tissue), chondroblasts (cartilage), and osteoblasts (bone)

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4
Q

Collagen type I distribution in what tissues

A

bone, tendon, skin, dentin, fascia, arteries

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5
Q

Collagen type II distribution in what tissues

A

cartilage, vitreous humor

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6
Q

Collagen type III distribution in what tissues

A

skin, blood vessels, uterus

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7
Q

Collagen type IV distribution in what tissues

A

basement membrane

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8
Q

Collagen type V distribution in what tissues

A

skin, placenta, blood vessels, chorion uterus

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9
Q

Synthesis of collagen

A

INTRACELLULAR STAGE consists of the production of procollagen from precursor polypeptide chains that undergo the sequence of hydroxylation (requires vit C), glycosylation (galactosyl and glucose sugars added to hydroxylysyl residues), formation of a triple helix, and secretion. The hydroxylation requires vitamin C EXTRACELLULAR STAGE consists of the conversion of procollagen to tropocollagen by limited proteolysis from the amino and carboxyl termini, self-assembly of tropocollagen molecules into fibrils, and finally cross-linking of the fibrils to form collagen fibers facilitated by lysyl oxidase requiring copper cofactor

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10
Q

in collagen the a.a. acid arrangement must have

A

every third a.a. must be glycine

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11
Q

Types of Ehlers-Dalos syndrome

A

6 major types: hypermobility, classical, vascular, kyphoscoliosis, arthrochalasia, dermatospraxis

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12
Q

Osteogenesis imperfecta due to

A

lack of type I collagen

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13
Q

Scurvy due to

A

vitamin c deficiency, unable to hydroxylate lysine and proline

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14
Q

collagen cross-linking occurs by

A

derived from lysyl and hydroxylysyl amino acid residues. First, oxidative deamination of the -NH2 group of two amino acids forming corresponding aldehyde-containing residues allysine, and hydroxyallysine. Second aldehyde groups react spontaneously with each other forming collagen cross links. first reaction is copper dependent and occurs extracellularly by lysyl oxidase

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15
Q

Elastin

A

Connective tissue protein, extensively interconnected, rubbery network resulting in connective tissue elasticity

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16
Q

Elastin rich in

A

proline and lysine

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17
Q

elastic fibers composed of

A

elastin & glycoprotein microfibrils

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18
Q

Elastin can be found in

A

lungs, the walls of large arteries, and elastic ligaments

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19
Q

Structure of elastin

A

Tropoelastin interact with fibrillin (defects causes marfan syndrome), desmosine cross-link by lysyl oxidase

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20
Q

Role of alpha 1-antitrypsin in elastin degradation

A

protease inhibitor, >90% of plasma alpha 1-globin fraction, major role of inhibiting neutrophil elastase, synthesized in the liver, monocytes, alveolar macrophages

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21
Q

Role of alpha 1 antitrypsin in the lungs

A

inhibit elastase to prevent alveolar wall damage destruction that would lead to emphysema

22
Q

Emphysema resulting from alpha 1 antitrypsin deficiency

A

2~5% of emphysema patients due to genetic defects, autosomal recessive inheritance GAG->AAG (Glu->Lys) at 342 (E342K): the most widespread

23
Q

Smokers with alpha 1 antitrypsin deficiency

A

inactivate Met of alpha 1 antitrypsin

24
Q

alpha 1 antitrypsin deficiency treatment

A

weekly IV administration of alpha 1 antitrypsin

25
Q

Marfan syndrome is

A

Hereditary disorder of the connective tissues A defect in fibrillin gene

26
Q

Marfan characterized by

A

Typically very tall slender, loose jointed, long bones of skeleton, arms, legs, fingers, & toes Heart & blood vessel abnormalities No cure, many options to manage the symptoms

27
Q

Proteoglycans (mucopolysaccharides)

A

Consist of long linear chains of glycosaminoglycans attached to a core protein. Each chain is composed of a repeating disaccharide that usually is negatively charged and contains a hexoseamine and a uronic acid

28
Q

Proteoglycans are major components of

A

connective tissue and participate with other structural constituents, collagen and elastin in the organization of the extra cellular matrix

29
Q

Mucopolysaccharidoses:

A

genetic disorders of proteoglycan metabolism, characterized by excessive accumulation and excretion of glycosaminoglycans

30
Q

Cause of mucopolysaccharidoses

A

deficiencies of lysosomal enzymes that are responsible for the degradation of mucopolysaccharides

31
Q

Hyaluronate GAG composition

A

Amino sugar: D-Glucosamine Uronic acid: D-Glucuronate

32
Q

Chondroitin sulfate GAG composition

A

Amino sugar: D-Galactosamine Uronic acid: D-Glucuronate

33
Q

Dermatan sulfate GAG composition

A

Amino sugar: D-Galactosamine Uronic acid: L-Iduronate, D-Glucuronate

34
Q

Keratan sulfate GAG composition

A

Amino sugar: D-Glucosamine Uronic acid: none (but contains D-Galactose)

35
Q

Heparan sulfate GAG composition

A

Amino sugar: D-Glucosamine Uronic acid: D-Glucuronate (major), L-Iduronate (minor)

36
Q

heparin GAG composition

A

Amino sugar: D-Glucosamine Uronic acid: L-Iduronate (major), D-Glucuronate (minor)

37
Q

MPS I H

A

Hurler

38
Q

MPS I S

A

Scheie

39
Q

MPS I H/S

A

Hurler/Scheie

40
Q

MPS II (severe)

A

Hunter (severe)

41
Q

MPS II (mild)

A

Hunter (mild)

42
Q

For all mucopolysaccharidoses GAG affected

A

Dermatan sulfate, heparin sulfate

43
Q

Scheie enzyme deficiency

A

alpha-L-Iduronidase

44
Q

Hurler/Scheie enzyme deficiency

A

alpha-L-Iduronidase

45
Q

Hunter (severe) enzyme deficiency

A

Iduronate sulfatase

46
Q

Hunter (mild) enzyme deficiency

A

Iduronate sulfatase

47
Q

Hurler enzyme deficiency

A

alpha-L-Iduronidase

48
Q

Peptidoglycan

A

Components of bacterial cell walls Consist of heteropolysaccharide chains cross-linked by short peptide chains Bear antigenic determinants

49
Q

Peptidoglycan synthesis

A

target for the action of the penicillins and cephalosporins

50
Q

Gram positive cell walls consist of

A

thick peptidoglycan and polyol phosphate polymer known as teichoic acids

51
Q

Gram negative cell walls consist of

A

peptidoglycan and outer membrane system external to the plasma membrane, no teichoic acids

Biochem Fall (13 decks)

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