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Biochem > ECM/Proteoglycans Deevska > Flashcards

ECM/Proteoglycans Deevska Flashcards

1
Q

Vitamin C is required as a cofactor for the
enzymes involved in __________ of
______ and___________residues in collagen

A

hydroxyla8on

proline and lysine

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2
Q

Vitamin C:

lack of proline and lysine
hydroxyla8on lead to ________________
which prevents the forma8on of a __________________
and affects

A

impairment of the interchain H-­‐bond forma8on

stable triple helix

proper cross-­‐linking

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3
Q

Vitamin C deficiency symptoms (scurvy)

These greatly decrease the tensile
strength of the assembled fiber

  •   Easy _____
  •   Loose ___________
  •   Poor
  •   Poor
A

bruising

teeth and bleeding gums

wound healing

bone development

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4
Q

Colleen fibers are rich in what two AA?

A

Proline

Glycine

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5
Q

Contains hydroxyproline and
hydroxylysine formed by post-­‐
transla8onal hydroxyla8on of proline and
lysine residues:

A

Collagen

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6
Q

Hydroxylysine may be glycosylated with
glucose or galactose before the forma)on
of the triple helix

A

Collagen

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7
Q

Elastin is rich in what two AA?

A

Rich in proline and lysine but contains scant

hydroxyproline and hydroxylysine

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8
Q

Ø  Extensively interconnected, rubbery
network that can stretch and bend in any
direc8on when stressed, giving connec8ve
8ssue elas8city

A

Elastin

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9
Q

Proteoglycans :

Large complexes of 
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
associated with a \_\_\_\_\_\_\_\_\_\_\_\_\_
~\_\_\_\_%carbohydrate 
~\_\_\_\_\_\_\_% protein
A

heteropolysaccharide chains

small
amount of protein (“core
protein”)

95

5

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10
Q

Glycoproteins
Protein with a variable___________ but typically small amount of
(2 to 10 sugar residues)

A

carbohydrate

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11
Q

Chondrodystrophy

A

A defect in the sulfa8on of the growing glycosaminoglycan chains
results in one of several disorders (chondrodystrophies) that affect
the proper development and maintenance of the skeletal system.

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12
Q

Characteristics. Of. Chondrodystrophy

A

autosomal recessive disorders

•  Characteris)cs:
-­‐  Dwarfism -­‐ a normal-­‐sized trunk and abnormally shortened limbs
& extremi)es
-­‐  over 100 specific skeletal dysplasias
Occurrence:
1:25,000 births, male and female, all races

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13
Q

Mucopolysaccharidoses definition?

A

Progressive disorders characterized by glycosaminoglycans
accumula)ng in the lysosomes of various )ssues results in
oligosaccharides in the urine

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14
Q

Characteristics of Mucopolysaccharidoses

A

Hereditary diseases affec)ng 1:25,000 births

Ø  Autosomal recessive, except Hunter syndrome (which is X-­‐linked)

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15
Q

Diagnosis of Mucopolysaccharidoses

A

Diagnosis:
•  The specific mucopolysaccharidoses is determined by iden)fying the
structure present at the non-­‐reducing end of the oligosaccharide
•  Diagnosis is confirmed by measuring the pa)ent’s cellular level of
lysosomal hydrolases

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16
Q

Symptoms of Mucopolysaccharidoses

A

Symptoms:
•  apparently normal at birth, gradually deteriorate, severe cases may
result in childhood mortality
•  a range of skeletal and extracellular matrix deformi)es, and mental
retarda)on

17
Q

What two Mucopolysaccharidoses diseases cause corneal clouding?

A 
Hurler Syndrome (MPS 1 H) 
Sly Syndrome (MPS VII)
18
Q

Treatments for Hurler Syndrome (MPS 1 H)

A

Bone marrow or cord blood transplant
(preferably within 18 months of age)
•  Enzyme replacement therapy available

19
Q

Sly Syndrome (MPS VII) treatments

A

Treatments:
•  no approved specific treatments for MPS
VII

20
Q

Symptoms of Hurler Syndrome (MPS 1 H)

A 
Most severe form of MPS 
•  Corneal clouding 
•  Mental retarda)on, dwarfing 
•  Coarse facial features 
•  Upper airway obstruc)on 
•  Deposi8on in coronary artery  leads to 
ischemia and early death
21
Q

Symptoms of Hunter Syndrome (MPS II)

A 
Iduranate sulfatase deficiency (dermatan  
and heparan sulfates) 
•  X-­‐Linked 
•  Wide range of severity 
   -­‐ NO corneal clouding 
   -­‐ physical deformity 
   -­‐ mental retarda)on 
   -­‐ coarse facial features 
   -­‐ growth deficiency
22
Q

Symptoms of Sanfilippo Syndrome Types A-­‐D (MPS III)

A

Symptoms:
•  Nervous system disorders
•  Mental retarda8on

23
Q

Symptoms of Sly Syndrome (MPS VII)

A 
Symptoms: 
•  Corneal clouding 
•  Depressed nasal bridge, epicanthic folds 
•  long piltrum, thin upper lip 
•  hypertrichosis (abnormal hair growth)
24
Q

Treatments for Hunter Syndrome (MPS II)

A

Treatments:
•  Enzyme
replacement
therapy available

25
Q

Treatments for Sanfilippo Syndrome Types A-­‐D (MPS III

A

None listed

26
Q

Cause of I-cell disease

A

Deficiency in the ability to phosphorylate mannose: acid hydrolase
enzymes fail to target lysosomes and by the default secretory
pathway are trafficked outside of the cell and found in the plasma
Ø  Accumula8on of large lysosomal inclusion bodies composed of the
missing hydrolase substrate – lysosomal storage disease

27
Q

Symptoms of I cell-disease

A

Symptoms:
•  Skeletal abnormali)es, restricted joint movement, coarse
dysmorphic facial features, severe psychomotor impairment
•  Death likely prior to 8 years old

28
Q

Classification of GAGs: Chondroitin 4- And 6-Sulfates

Found in?

A

Most abundant GAGs in the body

Found in cartilage, tendons, ligaments, aorta

Form proteoglycan aggregates ; aggregates noncovalently with hyaluronic acid

In cartilage it binds collagen and hold fibers in a tight, strong network

29
Q

Classification of GAGs: Keratan Sulfates (KS) I and II

A

MOST heterogenous GAGS

KS I Found in corneas
KS II found in loose connective tissue proteoglycan aggregates with chondroitin sulfate

30
Q

Classification of GAGs: Hyaluronic acid( Hyaluronate)

A

:Found in synovial fluid of joints vitreous Humor of the eye, the umbilical cord, loose connective tissue and cartilage

Serves as a lubricant and shock absorber

Diff from other GAGs- unsulfated found in bacteria as well

31
Q

Classification of GAGs: Dermatan Sulfate

A

:Found n skin blood vessels and heart valves

32
Q

Classification of GAGs Heparin

A

Intracellular component of mast cells that line arteries, especially in liver lungs and skin

Serves as an anticoagulant

33
Q

Classification of GAGs Heparan sulfate

A

:Extracellular GAG found in basement membrane and as a ubiquitous component of cell surfaces

Biochem (4 decks)

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