Glycogen and Carb metaolism

Question 1

Glycogenin, Glycogen Synthase, Branching Enzyme

Answer 1

polymerization of glucose (glycogen synthesis)

Question 2

First step of glycogen synthesis

Answer 2

activate glucose

Question 3

Primer protein for glycogen synthesis

Answer 3

Glycogenin

Question 4

Transfers glucose from UDP-glucose to non-reducing ends forming alpha(1,4) glycosidic bonds

Answer 4

Glycogen Synthase (glycogen synthesis)

Question 5

Cleaves off short fragments from alpha (1,4) chains and connects to make alpha (1,6) linkages

Answer 5

Branching Enzyme (glycogen synthesis)

Question 6

Location of glycogen synthesis

Answer 6

cytosol

Question 7

Releases glucose-1-phosphate from glycogen

Answer 7

glycogen phosphorylase (glycogen degradation)

Question 8

Transfers branches and releases glucose

Answer 8

debranching enzyme (glycogen degradation)

Question 9

Beta cells of pancreas

Answer 9

insulin

Question 10

Alpha cells of pancreas

Answer 10

glucagon

Question 11

Hormone released from adrenal medulla

Answer 11

epinephrine

Question 12

Phosphorylation of glycogen phosphorylase

Answer 12

becomes active (glycogen degradation)

Question 13

Phosphorylation of glycogen synthesis

Answer 13

becomes inactive

Question 14

What organ synthesizes glycogen but do not respond to glucagon? They lack what enzyme that releases glucose to bloodstream?

Answer 14

muscles, lack glucose-6-phosphatase (G6P»Glucose)

Question 15

Main source of blood glucose before breakfast.

Answer 15

glucoNEOgenesis

Question 16

Main source of blood glucose during well-fed state.

Answer 16

glycogen degradation

Question 17

muscle weakness/ exercise intolerance

enlarged liver

Answer 17

general symptoms of glycogen storage disease

Question 18

defect in glucose-6-phosphatase

Answer 18

Type I GSD- van Gierke disease

inability to convert G6P to glucose for release to bloodstream

Question 19

acid maltase deficiency

Answer 19

Type II GSK - Pompe disease

glycogen accumulation due to deficiency in the enzyme that catalyzes glycogen breakdown in lysosomes

Question 20

defective glycogen debranching enzyme

Answer 20

Type III GSD - Cori disease

Question 21

defective glycogen phosphorylase in muscles

Answer 21

Type IV GSD - McArdle disease

unable to use stored glycogen

Question 22

buildup of NADH, CAC is inhibited, gluconeogenesis is inhibited (no pyruvate and OAA from CAC). NADH gets used up by lactate dehydrogenase and malate dehydrogenase

Answer 22

alcohol metabolism makes NADH

- causes lactic acidosis and hypoglycemia (inhibition of gluconeogenesis)

Question 23

gel-like component of ECM comes from what?

Answer 23

proteoglycans (acidic and basic)

Question 24

large molecules that consist mainly of acidic modified sugars

Answer 24

proteoglycans

Question 25

where does glycosylation of proteins occur?

Answer 25

ER and Golgi

Question 26

facilitate the excretion of poorly water-soluble metabolites

Answer 26

acidic carbohydrates are abundant in ECM

Question 27

N-acetylglucosamine added

Answer 27

Heparan Sulfate | Keratin Sulfate

Question 28

N-acetylgalactosamine added

Answer 28

Chondroitin Sulfate

Question 29

Both sugars added

Answer 29

Dermatan Sulfate | Heparin

Question 30

no sugar added

Answer 30

hyaluronic acid

Question 31

degradation of proteoglycans

Answer 31

in lysosomes

Question 32

proteins linked to long polymers of C-6 acids and aminosugars which are sulfated in the Golgi after synthsis

Answer 32

proteoglycans

Question 33

mannose, galactose, and fucose

Answer 33

required for glycosylation of proteins

Question 34

made by fructose-6-phosphate

Answer 34

mannose and fucose | - heavy regulation of PFK1 in glycolysis

Question 35

genetic disease | - defective proteoglycan-degrading hydrolases leading to skeletal deformation and intellectual disability

Answer 35

Mucopolysaccharidosis (MPS)

Question 36

Accumulation of dermatan sulfate and heparan sulfate caused by defective enzyme iduronate sulfatase - X-linked recessive

Answer 36

Hunter

Question 37

accumulation of dermatan sulfate and heparan sulfate caused by defective enzyme alpha-iduronase - auto-recessive

Answer 37

Hurler-Scheie

Question 38

accumulation of heparan sulfate caused by different defects of heparan sulfate degradation (A,B,C,D roots)

Answer 38

Sanfilippos's (A, B, C, D)

Question 39

occurs in the liver and require production of UDP-glucuronic acid to serve as a substrate

Answer 39

glycosylation

Question 40

increases water solubility and is a common path for the body to excrete poorly water-soluble molecules such as hemoglobin

Answer 40

glycosylation

Question 41

- glucuronate linked to a glucosamine and is the backbone of ECM - also allow newly synthesized proteoglycans to be integrated into the meshwork of ECM

Answer 41

hyaluronic acid

Question 42

- lack mitochondria thus do not undergo CAC or oxidative phosphorylation - depends on glycolysis for energy - can perform anaerobic reactions to produce lactate - utilizes Cori cycle - utilizes PPP for NADPH and/or ribose synthesis

Answer 42

RBCs

Question 43

- can perform glycogen synthesis and degradation - can fully metabolize glucose via glycolysis, CAC and ox. phos - perform PPP when needed - can perform anaerobic reactions to produce lactate

Answer 43

skeletal muscles

Question 44

- can perform glycogen synthesis and degradation - can fully metabolize glucose via glycolysis, CAC and ox. phos - perform PPP when needed - cannot perform anaerobic reactions to produce lactate

Answer 44

cardiac muscles

Question 45

- fully metabolize glucose - makes and degrades glycogen - performs Cori cycle - performs PPP if needed - does not perform anaerobic reactions

Answer 45

hepatocytes