Biochem Exam 2: Complex Carbs

Front 1

What are the four main groups of carbohydrates from smallest to largest?

Back 1

monosaccharides --> Disaccharides --> Oligosaccharides --> polysaccharides

Front 2

What carbohydrate group includes glucose, galactose, and fructose?

Back 2

monosaccharides

Front 3

Lactose is the main _____

Back 3

disaccharide

Front 4

_____ are short, non-repetitive complex carbohydrates which usually attach to lipids or proteins, making glycoproteins and glycolipids

Back 4

oligosaccharides

Front 5

_____ are large repetitive carbohydrates, such as glycogen and GAGs

Back 5

polysaccharides

Front 6

In order to link monosaccharides (sugars) together or attach them to proteins
or lipids, the monosaccharides have to be in a ______ form

Back 6

nucleotide-activated form

(necessary for building complex carbohydrates)

Front 7

Proteoglycans are composed of a protein core and (glycosaminoglycan/oligosaccharide) chains, while glycoproteins are composed of a protein core and (glycosaminoglycan/oligosaccharide) chains.

Back 7

glycosaminoglycan (GAG)

oligosaccharide

Front 8

Oligosaccharides can be (N/O)-linked and attach proteins to the hydroxyl group of Ser or Thr, or (N/O)-linked and attach to the amide group of Asn.

Back 8

O-linked (linear, made in golgi)

N-linked (branched, made in ER then golgi)

Front 9

______ are enzymes that catalyze the addition of monosaccharides to proteins, lipids or growing oligo/polysaccharide chains

Back 9

Glycosyltransferases

(necessary for building complex carbohydrates)

Front 10

Mucins are large glycoproteins found mainly where?

Back 10

digestive tract and airways

Front 11

Mucins aggregate to form networks. What do these networks provide?

Back 11

a physical barrier against pathogen invasion

Front 12

Glycosaminoglycans are repeating disaccharide units of ______

Back 12

an acidic sugar (uronic or iduronic acid) and an N-acetylated amino sugar

Front 13

All glycosaminoglycans are generally the same size, except _____, which can be larger

Back 13

hyaluronic acid

Front 14

All glycosaminoglycans (GAGs) are (positively/negatively) charged

Back 14

-negatively charged

Front 15

All GAGs but _____ are sulfated, attached to a protein core, and synthesized in the golgi. This is synthesized in the ____

Back 15

-hyaluronic acid
-plasma membrane

Front 16

Glycosaminoglycans negative charge attract ______, which makes them behave like "sponges". This makes them ______ under compressive force

Back 16

-lots of water
-confer resilience to tissues

Front 17

______ is essential for embryogenesis, morphogenesis and wound healing. It is also essential for vertebrate life

Back 17

Hyaluronic acid

Front 18

Hyaluronic acid is used for _____

Back 18

-eye surgery (Healon)
-osteoarthritic pain (intraarticular synvisc injection)

Front 19

Chondrotin sulfates can be sulfated in what positions of N-acetyl-galactosamine

Back 19

4th and 6th position of N-acetyl-galactosamine

Front 20

Chondrotin sulfates is mainly found in _____

Back 20

cartilage
(also found in other tissues, tendon, ligaments, aorta)

Front 21

What is the main glycosaminoglycan that aggregates with proteoglycans and is also the most abundant glycosaminoglycan in the body?

Back 21

chondrotin sulfates

Front 22

Accumulation of what glycosaminoglycan can lead to mitral valve prolapse (thickening and abnormal displacement of left atrium)

Back 22

dermatin sulfate

Front 23

This glycosaminoglycan is mainly found in skin, blood vessels and heart valves

Back 23

dermatin sulfate

Front 24

Keratan sulfates are the only GAGs that do not contain _____

Back 24

acidic sugar
(have galactose instead)

Front 25

What are the two types of keratin sulfates?
What are they linked to?
Where are they mainly found?

Back 25

KSI: linked to the (N) amide of Asn (mainly found in cornea)
KS II: linked to the hydroxyl of Ser (mainly found in cartilage)

Front 26

Undersulfated keratin sulfate in cornea leads to _____ deficiency and _____ as a defect

Back 26

-Carbohydrate sulfotransferase 6 (CHST6) deficiency
-macular corneal dystrophy

Front 27

Hyaluronic acid is mainly located in ______________________, _________________, and ______________________.

Back 27

cartilage
synovial fluid of joints
vitreous humor of the eye
(also in umbilical cord)

Front 28

Dermatan sulfate differs from chondroitin sulfate only in the orientation of the ___________________.

Back 28

carboxyl group

(both iduronic & glucuronic acids found in dermatan sulfate)

Front 29

Heparin and Heparan sulfate have very similar structure but differ in ___________

Back 29

sulfination pattern
(heparin is more sulfated)

Front 30

Heparan sulfate is a component of _______________ in basement membrane and ________________ on cell surfaces.

Back 30

proteoglycan (perlecan)

molecules/receptors

Front 31

Heparin is stored and released in _________ and used as a medicinal ___________________.

Back 31

mast cells

anticoagulant (managment of MI and deep vein thrombosis)

Front 32

____________ contains one of the highest concentration of GAGs in the human body, which provides the tissue with resilience under compressive forces. These GAGs are mainly __________ with some _______________.

Back 32

Cartilage

chondroitin sulfates with some keratan sulfate

Front 33

The main cartilage proteoglycan __________ contains ~100 GAG chains and has a characteristic _____________ structure.

Back 33

aggrecan
bottle-brush

Front 34

Aggrecan (noncovalently/covalently) interacts with ____________ and ______________.

Back 34

noncovalently

hyaluronic acid and link protein

Front 35

Excessive degradation of cartilage proteoglycans leads to defective cartilage function in what diseases? (3)

Back 35

osteoarthritis
rheumatoid arthritis
systematic lupus erythematosus (SLE)

Front 36

Steps of proteoglycan synthesis (5):

Back 36

1. synthesis of amino sugars (cystol)
2. synthesis of glucuronic acid (cystol)
3. synthesis of core protein (endoplasmic reticulum)
4. synthesis of GAG chain (golgi or plasma membrane for hyaluronic acid)
5. Sulfation (golgi)

Front 37

Amino sugars derive from ___________ and must be in _______ form to be added to the GAG chain. _________ provides the amino group for amino sugar synthesis.

Back 37

glucose

UDP-linked form

Glutamine

Front 38

Glucuronic acid is synthesized from ______________ by oxidation and must also be in ________form to be added to the GAG chain

Back 38

UDP-glucose

UDP-linked form

Front 39

GAG chains are mainly synthesized on _____________ of the core protein. ___________________ enzymes add UDP-sugars to produce growing chain.

Back 39

serine residues

Glycosyltransferase

Front 40

_______________enzymes add sulfate groups to the GAG chains. The sulfate donor is ___________.

Back 40

Sulfotransferase

PAPS (3'-phosphoadenosyl-5'-phosphosulfate)

Front 41

Extracellular GAGs are taken into cell via _____________, then transported to ________________ for degradation.

Back 41

endocytosis

lysosomes

Front 42

________________________, a group of lysosomal storage diseases are caused by ______________________.

Back 42

Mucupolysaccaridoses

deficient lysosomal degradation

Front 43

2 main functions of glycoproteins:

Back 43

1. binding site for pathogens
2. blood group antigens

Front 44

Hurlers syndrome is a _____________________ deficiency that affects the degradation of _______________ and _______________. Symptoms include corneal clouding mental retardation, dwarfing, coarse facial features, upper airway obstruction.

Back 44

alpha-Liduronidase

dermatan sulfate and heparan sulfate

Front 45

Hunters syndrome is a _____________________ deficiency that affects the degradation of ________________ and _____________. Symptoms include mild to severe physical deformity and mental retardation. (no corneal clouding)

Back 45

Iduronate sulfatase

dermatan sulfate and heparan sulfate

Front 46

Sanfilippo syndrome has a variety of forms with different affected enzymes, all of which affect ____________. Symptoms include severe nervous system disorders and mental retardation.

Back 46

heparan sulfate

Front 47

What enzymes do the following types of Sanfilippo Syndrome effect?
Type A:
Type B:
Type C:
Type D:

Back 47

Type A: Heparan sulfamidase
Type B: N-Acetylglucosaminidase
Type C: Glucosamine-N-acetyltransferase
Type D: N-Acetylglucosamine 6-sulfatase

Front 48

Sly syndrome is a _________________ deficiency that affects degradation of _______________ and _________________. Symptoms include hepatosplenomegaly, skeletal deformity, short stature, corneal clouding, mental deficiency.

Back 48

beta-Glucuronidase

dermatan sulfate and heparan sulfate

Front 49

I-cell disease is caused by the deficiency of the ability to _____________, which is necessary for the delivery of lysosomal enzymes that degrade macromolecules. Characterized by skeletal abnormalities, restricted joint movement, coarse facial features, and severe psychomotor impairment.

Back 49

phosphorylate mannose

Front 50

Complex carbohydrates participate in pathogenic infections via viral entry and release. Influenza virus binds to ____________ on cell surface glycoproteins. HxNy refers to ____________ which facilitates viral entry, and _________ which facilitates release of new viruses from infected cells.

Back 50

sialic acids

hemagglutinin

neuraminidase (targeted by tamiflu- competitive inhibitor)

Front 51

What are the main components of the extracellular matrix (5)?

Back 51

collagens
elastic fibers
proteoglycans
hyaluronan
glycoproteins

Front 52

On proteoglycans, GAGs are attached to the protein core via a specific linkage region. This region can be attached to what 2 locations?

Back 52

either a hydroxyl of Ser of amid of Asn

Front 53

In I-cell disease, lysosomal enzymes are secreted instead of delivered to the lysosomes. This prevents macromolecules from being degraded, leading to ________ and the formation of ___________.

Back 53

undegraded macromolecules lead to enlarged lysosomes and the formation of inclusion bodies

Front 54

Lysosomes are essential for intracellular degradation of macromolecules. Delivery of lysosomal enzymes into the lysosomes requires what?

Back 54

phosphorylation of mannose on an N-linked oligosaccharide of the enzyme