Glycogenesis Flashcards

1
Q

The glycogen synthesis occurs by a pathway similar to the reversal of glycogen breakdown

T/F

A

F

distinctly different from Glycogenesis

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2
Q

Glycogenesis: Activation of Glucose

UDP glucose is formed from _____ and ____ by the enzyme _________

A

glucose-1-phosphate and UTP

UDPglucose pyrophosphorylase.

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3
Q

Full meaning of UTP is _______

A

uridine triphosphate

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4
Q

Glycogenesis: Glycogen Synthase

The _____ moiety from UDP-glucose is transferred to a ______ (_____) molecule. The primer is essential to accept the glycosyl unit. The primer is made up of a ____-____ complex.

In the next step,_________ are sequentially added by the enzyme ________

The glucose unit is added to the _______ ((outer or inner?) ) end of the glycogen primer to form an ______ glycosidic linkage and UDP is liberated.

A

glucose

glycogen primer

glycogenin

protein-carbohydrate

activated glucose units

glycogen synthase

nonreducing ; outer

alpha-1,4

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5
Q

Glycogenin is a ____ protein, having ___ identical monomers.

An oligosaccharide chain of _____ units is added to each monomer.

A

dimeric

two

7 glucose

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6
Q

Glycogenesis: Branching Enzyme

The glycogen synthase can add glucose units only in ____ linkage.

A branching enzyme is needed to create the ____ linkages.

When the chain is lengthened to ____ glucose residues, the branching enzyme will transfer a block of _____ glucose residues from this chain to another site on the growing molecule.

The enzyme amylo[1,4]→[__]_____ (branching enzyme) forms this alpha-1,6 linkage

To this newly created branch, further glucose units can be added in alpha- ___ linkage by _______

A

alpha-1,4

alpha-1,6

11 - 12

6 to 8

1,6; transglucosidase

1,4; glycogen synthase.

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7
Q

The phosphorylated form of glycogen phosphorylase is (active or inactive ?)

glycogen synthase becomes (active or inactive?) on phosphorylation.

A

Active

Inactive

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8
Q

Effect of ATP on

glycogen phosphorylase (liver) 
glycogen phosphorylase (muscle ) 
Glycogen synthase (liver)
Glycogen synthase (muscle )
A

Inhibit

Inhibit

Inhibit

Inhibit

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9
Q

Effect of AMP on

glycogen phosphorylase (liver) 
glycogen phosphorylase (muscle ) 
Glycogen synthase (liver)
Glycogen synthase (muscle )
A

Activation

Activation

Lol

Lol

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10
Q

Effect of glucose 6 phosphate on

glycogen phosphorylase (liver) 
glycogen phosphorylase (muscle ) 
Glycogen synthase (liver)
Glycogen synthase (muscle )
A

Lol
lol

Activate

Activate

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11
Q

Effect of calcium on

glycogen phosphorylase (liver) 
glycogen phosphorylase (muscle ) 
Glycogen synthase (liver)
Glycogen synthase (muscle )
A

Activate

Activate

Lol

Lol

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12
Q

von Gierke’s disease

______ enzyme is deficient

Xterized by :

______ ——-glycemia; _____megaly

A

Glucose-6-phosphatase

Fasting hypo

hepato

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13
Q

Limit dextrinosis aka ____ disease

_____ enzyme is deficient

Xterized by:——— accumulates; _____ ——-glycemia; ____megaly

A

Cori’s

Debranching enzyme

Highly branched dextrin

Fasting hypo

Hepato

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14
Q

Amylopectinosis aka ______ disease

______ enzyme is deficient

Xterized by: Glycogen with _______ ; ________megaly; ___ ——glycemia; death by age of ___

A

Anderson’s

Branching

few branches

hepatospleno

mild hypo

5

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15
Q

McArdle’s disease

______ enzyme is deficient

Xterized by :_____ intolerance; accumulation of ______ in muscles

A

Muscle phosphorylase

Excercise

glycogen

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16
Q

Hers’ disease

_______ enzyme is deficient

Xterized by : _____ ___glycemia; _____megaly; (better or worse?) prognosis than other types

A

Liver phosphorylase

Mild hypo

hepato

better

17
Q

The ____ cycle is the first metabolic pathway to be elucidated

A

urea

18
Q

The urea cycle is known as _______ urea cycle.

A

Krebs–Henseleit

19
Q

Urea cycle

As ——- is the first member of the reaction, it is also called as _____ cycle.

A

ornithine

Ornithine

20
Q

The two nitrogen atoms of urea are derived from two different sources, one from _____ and the other directly from the alpha amino group of _______.

A

ammonia

aspartic acid

21
Q

Urea cycle

Step 1

One molecule of ______ condenses with ___ in the presence of ___ molecules of ATP to form __________.

The reaction is catalysed by the (mitochondrial or cytoplasmic?) enzyme ________________.

A

ammonia

CO2

two

carbamoyl phosphate

Mitochondrial

carbamoyl phosphate synthetase-I (CPS-I)

22
Q

_____ reaction is the rate-limiting step in urea formation. It is irreversible and allosterically regulated.

A

CPS-I

23
Q

Urea cycle : Formation of Citrulline

The second reaction is (mitochondrial or cytoplasmic?) .

The _____ group is transferred to the NH2 group of _____ by _______________

A

Mitochondrial

carbamoyl

ornithine

ornithine transcarbamoylase (OTC)

24
Q

In urea cycle

The citrulline leaves the ____ and further reactions are taking place in ______.

A

mitochondria

cytoplasm

25
Q

Citruline is present or absent in

tissue proteins

milk

blood.

A

Absent

Present

Absent

26
Q

Urea cycle: Formation of Argininosuccinate

One molecule of ______ adds to citrulline forming a ____-______ bond which provides the 2nd nitrogen atom of urea. __________ catalyses the reaction

This needs hydrolysis of ___ to ____ level, so two high energy phosphate bonds are utilized.

A

aspartic acid

carbon to nitrogen

Argininosuccinate synthetase

ATP to AMP

27
Q

The PPi is an (activator or inhibitor?) of Formation of Argininosuccinate
In urea cycle

A

Inhibitor

28
Q

Urea cycle : Formation of Arginine

Argininosuccinate is cleaved by _______ to _____ and _____.

The enzyme is inhibited by _____. But this is avoided by the ______ of the enzyme.

The fumarate formed may be funnelled into TCA cycle to be converted to ____ and then to _____ to be transaminated to _____

A

argininosuccinate lyase

arginine and fumarate
fumarate

cytoplasmic localization

malate; oxaloacetate; aspartate

29
Q

Thus the urea cycle is linked to TCA cycle through _______.

The 3rd and 4th steps taken together may be summarized as: Citrulline + aspartate → ________ +______

A

fumarate

Arginine ; fumarate

30
Q

Urea cycle: Formation of Urea

The final reaction of the cycle is the _____ of _____ to urea and ____ by ______

A

hydrolysis of arginine

ornithine

arginase

31
Q

The ornithine from urea cycle returns to the _____ to react with another molecule of _____ so that the cycle will proceed.

Thus, ornithine may be considered as a catalyst which enters the reaction and is regenerated.

A

mitochondria

carbamoyl phosphate

32
Q

The urea cycle consumes ___ high energy phosphate bonds

A

4

33
Q

The urea cycle and TCA cycle are interlinked, and so, it is called as “____________”.

A

urea bicycle