Term
1. collagen
2. elastin
3. glycoproteins
4. proteoglycans |
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Definition
| components of the extracellular matrix |
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Term
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Definition
| fibrous protein with low water solubility, long cylindrical shape, structural role, and greatest abundance in the body. |
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Definition
| Made up of mostly carbohydrates, with some protein modifications. Have repeating structures. |
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Term
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Definition
| basic structural unit of collagen, extractable from young animal tissue. |
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Term
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Definition
| has three peptide chains and 1,000 a.a. residues |
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Term
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Definition
[alpha1(I)]2 alpha2(I)
bone, skin, tendon, and cornea |
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Term
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Definition
[Alpha1(II)]3
cartilage, intervertebral discs, and vitreous body |
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Definition
[Alpha1(III)]3
fetal skin, cardiovascular system |
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Term
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Definition
[alpha1(IV)]3 OR [alpha2(IV)]3
basement membranes (glomeruli in kidney), lens capsule |
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Term
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Definition
| gets oxidized to dehydroascorbate (and peed out) in order to maintain iron in the reduced Fe2+ (ferrous) form. |
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Term
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Definition
| Most abundant amino acid in collagen. 1 out of 3. |
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Term
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Definition
| also abundant a.a. in collagen (~100 total) |
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Term
Hyp (4-hydroxyproline)
Hyl (5-hydroxylysine) |
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Definition
| Unusual amino acids in collagen containing an OH group added by post-translational modification |
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Term
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Definition
| recurring tripeptide sequence found in all collagens |
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Term
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Definition
prolyl residue + alphaKG + ascorbate -->
4-Hyp + succinate + CO2
(prolyl residue must be on AMINO side of a glycyl residue) |
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Term
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Definition
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Term
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Definition
| glycosylation of Hyl residues takes place where? |
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Term
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Definition
| 4-OH of this hexose sugar points up. |
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Term
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Definition
| 4-OH of this hexose sugar points down. |
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Term
Galactosyltransferase
Glucosyltransferase |
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Definition
| Enzymes that glycosylate Hyl before helix formation can occur |
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Term
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Definition
length = 3,000 A
diameter = 15 A
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Term
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Definition
Left handed helix.
Rise/residue (d) = 3.12 A
residues/turn (n) = 3
Pitch (rise/turn) (p) = 9.36 A |
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Term
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Definition
d = 1.5 A
n = 3.61
p = 5.4 A |
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Term
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Definition
H-bonds are longitudinal, intrachain, between backbone
carbonyl 'O' and amide 'H' |
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Term
| steric repulsion of pyrrolidone rings |
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Definition
| stabilizing force in tropocollagen helices |
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Term
| triple stranded mature collagen helix |
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Definition
Right handed superhelical cable
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Term
| triple stranded collagen helix |
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Definition
d = 2.9 A
n = 3.3
p = 9.5 A |
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Term
| triple stranded collagen helix |
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Definition
| Interstrand H-bonds run transversely between backbone 'NH' groups of Gly and backbone 'CO' groups. |
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Term
| triple helix: (Pro-Pro-Gly)n |
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Definition
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Term
| triple helix: (Pro-Hyp-Gly)n |
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Definition
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Term
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Definition
| precursor of tropocollagen |
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Term
| N-terminal peptides of procollagen |
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Definition
part of procollagen that contains intrachain disulfide bonds.
MW = 15kD |
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Term
| C-terminal peptides of procollagen |
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Definition
part of procollagen that contains intra- and interchain disulfide bonds as well as mannose rich oligosaccharides.
MW = 30kD |
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Term
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Definition
| conversion of pro-alpha1(I) to alpha1(I) changes the MW from ____ to ____ |
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Term
| Ehlers Danlos Syndrome type VII |
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Definition
| hypermobile joints, stretchy skin, short stature. |
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Term
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Definition
| deficient enzyme in Ehlers Danlos syndrome type VII |
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Term
| side by side, head to tail, quarter staggered. |
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Definition
| how do collagen fibrils arrange? |
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Term
diameter = 5,000 A
striation = 680 A (quarter staggered)
forms mature collagen by cross linking |
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Definition
| dimensions of a collagen fibril |
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Term
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Definition
Lysyl residues on different peptides + Cu + B6 ---->
Allysine (aldehyde) ----->
two allysines connect and form cross-link |
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Term
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Definition
| enzyme used in aldol cross link formation |
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Term
| Schiff base (aldimine) and lysinorleucine cross-links |
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Definition
lysine + allysine -->
Schiff base (aldimine) which is not permanent until reduced -->
Lysinorleucine (like leucine but no branching) |
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Term
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Definition
| fragile collagen in animals caused by feeding them sweet peas. |
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Definition
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Definition
| enzyme inhibited in lathyrism |
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Term
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Definition
| transcription occurs where? |
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Term
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Definition
| where polypeptide synthesis occurs |
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Term
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Definition
| where hydroxylation and glycosylation occur |
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Term
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Definition
| where procollagen (triple helix) formation occurs |
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Definition
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Term
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Definition
| where tropocollagen formation occurs |
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Term
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Definition
| where collagen fibril formation occurs |
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Term
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Definition
| where formation of cross-links to yield mature collagen fibrils occurs |
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Term
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Definition
| known as brittle bone disease or Lobstein syndrome |
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Term
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Definition
| stems from abnormalities in the collagen molecule formation in bone matrix. |
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Term
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Definition
| disorder of Type 1 collagen, of autosomal dominant inheritance, due to a mutation of structural genes for peptides of collagen |
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Definition
| bind water and cations to resist compaction. |
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Term
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Definition
| composed of glycosaminoglycans (GAGs) in repeating sequences: hexosamine-urate-hexosamine-urate..... |
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Term
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Definition
| a GAG (mucopolysaccharide) containing GlcUA, GlcNAc |
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Term
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Definition
| a GAG containing GlcUA, GalNAc-6S or GalNAc-4S |
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Term
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Definition
| GAG containing IdUA, GalNAc-4S |
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Term
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Definition
| GAG containing Gal, GlcNAc-6S |
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Term
| heparin and heparan sulfate |
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Definition
| GAG containing GlcUA-2S, GlcNS-6S |
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Term
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Definition
| GAG with three sulfate groups |
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Term
| heparan sulfate AND dermatan sulfate |
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Definition
| GAG with some iduronate in place of glucuronate |
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Term
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Definition
| lysosomal storage diseases with an invariable feature: dysostosis multiplex |
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Term
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Definition
| enzyme deficient in mucopolsaccharidoses |
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Term
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Definition
enzyme def: alpha-L-iduronase
urinary GAG excretion: DS and HS
serious mucopolysacchardosis where victims die before 10 |
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Term
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Definition
enzyme def: alpha-L-iduronase
urinary GAG excretion: DS and HS
mild mucopolysacchardiosis where victimes live past 10 |
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Term
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Definition
enzyme def: alpha-L-iduronase
urinary GAG excretion: DS and HS
intermediate severity mucopolysaccharidosis |
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Term
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Definition
enzyme def: iduronate sulfatase (removes the 2-S from IdUA)
urinary GAG excretion: DS and HS |
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Term
| Sanfilippo syndrome A form |
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Definition
enzyme def: heparan N-sulfatase (N-sulfamidase)
urinary GAG excretion: HS only |
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Term
| Tissue Plasminogen Activator (TPA) |
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Definition
this compound (bound to fibrin) catalyzes:
plasminogen ---> plasmin
which leads to:
fibrin clot --> soluble fibrin fragment |
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