Term
1. collagen
2. elastin
3. glycoproteins
4. proteoglycans |
|
Definition
| list the 4 components of extra-cellular matrix |
|
|
Term
|
Definition
| fibrous protein, abundant secondary structure, long cyllindrical shape, low water solubulity, structural rather than dynamic role |
|
|
Term
| repeating structures that are non-covalenly linked |
|
Definition
| what does abundant secondary structure mean? |
|
|
Term
|
Definition
|
|
Term
|
Definition
| mostly protein, modified with carbs, no repeated structures, component of ECM |
|
|
Term
|
Definition
| 25% of all protein in the boy, 74% of protein in skin |
|
|
Term
|
Definition
| component of ECM, mostly carbs modified by proteins (less protein that others) |
|
|
Term
|
Definition
| basic structural unit of collagen |
|
|
Term
|
Definition
| more easily extractable from tissues of young animals, because older animals have more cross-linking |
|
|
Term
|
Definition
| avg molecular wt of an amino acid |
|
|
Term
alpha1(I) x 2, alpha2(I) x 1
bone, skin, tendon, cornea |
|
Definition
| what is collagen type I composition and distribution |
|
|
Term
alpha1(II) x 3
cartilage, intervretebral disc, vitreous body |
|
Definition
| what is collagen type II composition and distribution |
|
|
Term
alpha1(III) x 3
fetal skin, cardiovascular system |
|
Definition
| what is collagen type III composition and distribution |
|
|
Term
alpha1(IV) x 3 or alpha2(IV) x 3
basement membrane, lens capsule |
|
Definition
| what is collagen type IV composition and distribution |
|
|
Term
|
Definition
| how many peptide chains in tropocollagen |
|
|
Term
|
Definition
| most abundant aa in collagen (first 2) |
|
|
Term
Hyp (4-hydroxyproline)
Hyl (5-hydroxylysine) |
|
Definition
| unusual amino acids found in collagen |
|
|
Term
|
Definition
| what is the recurring tri-peptide sequence in all collagens? |
|
|
Term
|
Definition
|
|
Term
|
Definition
| structure of 5-hydroxy lysine |
|
|
Term
|
Definition
|
|
Term
| must be on amino side of glycyl residue |
|
Definition
| where must the prolyl residue be to undergo 4-hydroxylation to form Hyp? |
|
|
Term
| no, only the ones on the amino side of glycyl |
|
Definition
| is free proline a substrate for prolyl hydroxylase? |
|
|
Term
| iron in Fe2+ ferrous, reduced state (need vitamin C for this -ascorbate) |
|
Definition
| what is present at the enzyme active site of prolyl hydroxylase and what is needed to keep it in the reduced state? |
|
|
Term
Scurvy, the integrity of blood vessels are compromised due to improper/insufficient collagen formation
causing bloody gums |
|
Definition
| what is hypovitaminosis C, and how does it present? |
|
|
Term
|
Definition
| what does the equation for Hyp formation look like? |
|
|
Term
| galactosyl transferase and glucosyl transferase |
|
Definition
| what enzyme is required for hydroxylysine glycosylation? |
|
|
Term
|
Definition
| when does glycosylation of Hyl residues occur relative to helix formation? |
|
|
Term
|
Definition
| most common type of constituent peptide in tropocollagen |
|
|
Term
| dehydroascorbate, it is a non-specific antioxidant that removes oxygen. it is peed out |
|
Definition
| what does ascorbate become after oxidation, and what happens if it builds up? |
|
|
Term
| collagen is more tightly wound(d= 3.3 coll vs 3.61 aplha), and more extended (pitch)9.5 vs 5.4 alpha |
|
Definition
| compare collagen to a right handed aplha helix |
|
|
Term
| steric repulsion of pyrrolidone rings of proline residues (no intra chain hydrogen bonds like alpha helix) |
|
Definition
| what are the stabilizing forces of tropocollagen? |
|
|
Term
1. interstrand hydrogen bonds
2. hydrogen bonds between water and Hyp |
|
Definition
| what are the stabilizing forces of mature collagen? |
|
|
Term
donor: backbone NH groups of Gly
acceptor: backbone CO groups
direction = transverse |
|
Definition
| what is the donor/acceptor of inter-strand hydrogen bonds of mature collagen and what direction? |
|
|
Term
| ppg has lower melting point(24) due to less H bonds phg has higher melting point(58) due to more H bonds |
|
Definition
| compare pro-pro-gly chains to pro-hyp-gly(like in tropocollagen) chains |
|
|
Term
n: intrachain disulfide bonds, collagen domain
C: inter + intra chain disulfide bonds
(sulfide bonds from cysteine S residues) |
|
Definition
| what structures exist at n terminal peptides of tropocollagen and at c terminal? |
|
|
Term
|
Definition
| where is the conversion of procollagen to tropocollagen? |
|
|
Term
| hypermobile joints, stretchable skin, short stature, due to deficiency of procollagen N-protease which cuts of n terminal peptide |
|
Definition
| what is Ehlers-Danlos syndrome Type VII |
|
|
Term
|
Definition
| tropocollagen fibrils associate and form collagen fibrils, how are they arranged? |
|
|
Term
| cross linking in mature collagen |
|
Definition
| what is the difference between mature collagen and collagen fibrils? |
|
|
Term
|
Definition
| draw the aldol cross-link reaction, what does it require? |
|
|
Term
| aldol cross links 2 lysyl residues by making them into 2 allysine (1 allysine link to 1 lysine) dependent on copper, b6 and oxygen |
|
Definition
| what is lysyl oxidase and what is it dependent on? |
|
|
Term
| lysine + allysine -> shiff base (aldimine) -> (reduced) lysinorleucine double bond is lost between nitrogen and carbon |
|
Definition
| what are the steps in the schiff base and lysinorleucine cross-link reactions? |
|
|
Term
| extremely fragile collagen in animals caused by ingestion of the seeds of sweet pea (Lathyris odoratus). the toxic agent = beta aminopropionitrile which inhibits lysyl oxidase (the cross linking enzyme) |
|
Definition
|
|
Term
1. transcription(nucleus)
2. polypeptide synthesis (in cyto with ER)
3. hydroxylation/glycosylation (in ER)
4. procollagen(triple helix formatin in ER) |
|
Definition
| what are the first 4 steps of collagen formation? |
|
|
Term
5. secretion (golgi)
6. tropocollagen formation (extracellular)
7. collagen fibril formation (extracellular)
8. formation of cross-links to yield mature collagen fibrils (extracellular |
|
Definition
| what are the last 4 steps of collagen formation? |
|
|
Term
aka brittle bone disease/lobstein syndrome. bone fragility stemming from abnormalities in collagen molecule forming the bone matrix. disorder of type 1 collagen. mostly autosomal dominant
molecular defect: mutations confined to structural genes for constituent peptides of collagen |
|
Definition
| what is osteogenesis imperfecta? |
|
|
Term
|
Definition
| 95% polysaccharide, 5%protein, polyanions that bind water and cations. |
|
|
Term
| glycosaminoglycans (GAGs) or mucopolysaccharides |
|
Definition
| what is the main component of proteoglycans? |
|
|
Term
hexosamine: glucosamine or galactosamine
uronic acid: beta d glucuronate or alpha-l-iduronate |
|
Definition
| what are the 2 main components(monmers) of GAGs |
|
|
Term
| cushion against mechanical impact, they associate with water molecules |
|
Definition
| what is the function of proteoglycans? why do they weigh more? |
|
|
Term
1. hyaluronic acid
2. chondroitin sulfates
3. dermatan sulfate
4. keratan sulfate
5. heparin and heparan sulfate |
|
Definition
| what are the 5 classes of glycosaminoglycans? |
|
|
Term
| hyaluronic acid found in synovial fluid |
|
Definition
| what is the most abundant GAG? |
|
|
Term
| structure of chondroitin 6 sulfate |
|
Definition
|
|
Term
| structure of dermatan sulfate |
|
Definition
|
|
Term
| structure of karatan sulfate |
|
Definition
|
|
Term
|
Definition
|
|
Term
| structure of heparin/heparan sulfate |
|
Definition
|
|
Term
|
Definition
| what are the 2 components(sugar/acid) of hyaluronic acid? |
|
|
Term
|
Definition
| what are the 2 components(sugar/acid) of chondroitin sulfate? |
|
|
Term
|
Definition
| what are the 2 components(sugar/acid) of dermatan sulfate? |
|
|
Term
|
Definition
| what are the 2 components(sugar/acid) of keratan sulfate? |
|
|
Term
|
Definition
| what are the 2 components(sugar/acid) of heparin, heparan sulfate? |
|
|
Term
1.HS has more N acetate, fewer sulfate groups (couterintuitive)
2. lower degree of O-sulfation
3. some iduronic acid in place of glucuronic acid |
|
Definition
| what are the 3 main differenes between heparin and heparan sulfate? |
|
|
Term
|
Definition
| what is the name for the lysosomal storage diseases? |
|
|
Term
|
Definition
| feature of all mucocpolysaccharidoses, name for morphologic abnormalities of many parts of the skeleton |
|
|
Term
| Hurler syndrome, DS and HS (remember the severity by Hurler syndrom makes you want to hurl, but scheie is okay) |
|
Definition
| most severe form of alpha l-iduronase deficient disorders. usually patients die before age 10. (What GAG is excreted also?) |
|
|
Term
| Scheie syndrome, DS and HS |
|
Definition
| mild form of alpha l iduronase deficiency, what is excreted? |
|
|
Term
| hurler-scheie syndrome HS and DS |
|
Definition
| intermediate severity form of alpha l iduronase enzyme deficiency, what is excreted? |
|
|
Term
| iduronate sulfatase, DS and HS |
|
Definition
| what enzyme is deficient in hunter syndrome and what is excreted? |
|
|
Term
| heparan n sulfatase(n-sulfamidase), only HS is excreted |
|
Definition
| what enzyme is deficient in Sanfillipo syndrom A-form, and what is excreted? |
|
|
