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Flashcards in Glucogenolysis Week 2 Deck (39):
1

Weight of glycogen in liver and muscle What are the glycogen reserves used for

10% of liver (maintain blood glucose) 1-2% of muscles (fuel reserve for ATP synthesis) Although since muscle is has a greater overall weight it has 2x the amount of glycogen

2

What is glycogenolysis

The break down of glycogen to glucose or glucose 6-P

3

When are glycogen levels high? and how long do they last

Highest after eating then slowly decreases.  

Glycogen last for 12-24 hours during fasting 

4

What sugar is in glycogen and what bonds does it have?

What organs would you find an what compartment of those organs

 

Only glucose and alpha 1-4 and alpha 1-6 (so glycogen is branched) 

Found in large amounts liver and muscle and most cells have some sort of glycogen in the cytosol

5

What is the main difference in glycogenolysis between the liver and every other cell? Dicuss function difference to!

In the liver Glycogen--> Glucose 6-P-- GLUCOSE (this is to balance blood gluose)

Every other cell--> Glucose 6-P--> GLYCOLYSIS so gnerates ENERGY 

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Pattern of Glycogen Content

After breakfast ectra glucose goes to storage so it's high. Slowly depletes between meals until it is restored. 

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What enzyme do muscle lack that makes glucose formation possible?

Muscle lacks glucose 6-phosphatase the enzyme that takes Glucose 6-p to glucose so most enter glycolysis 

 

** abouit 8% is converted to gree glucose**

11

Main differences in red and white muscle fibers 

Red muscles have good blood flow, myoglobin and PACKED with mitochondria. Glycogen to G-6-P-- Pyruvate --> CO2 and H2O (36 ATP because muscle use the glycerophophate shuttle)

 

White has poor blood supply, few mitochondria so the end product is lactate and have an enormous capability to glycogenolysis and glycolysis but only have short term full term capacity 

12

Structures of glycogen

Mainly linked by 1-4 glycosidic which in linear and branches 1-6 glycosidic linkage 

Branches occur every fourth glucosyl residue within the central cour and even less frequently further out

 

One starting point (reducing end/active) and we add glucose away towards non reducing ends

13

Why is glucose as glycogen

Energy could be stored as adipose but that isn't mobilized as fast, can't be converted without oxygen and can't maintain glucose levels

 

Can't be stored as glucose because that is osmotically active and ATP would have to be used agaisnt the glucose gradient. glycogen doesn't change osmolarity but glucose does

 

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Why is glycogen branched

More sites for degradation and synthessis so the process happens more quickly 

15

The first step in glycogenolysis

Glycogen phophosorlysis by glycogen phosphrylase (Pi is cleaved off the amylose chain) taking off one glucose at a time

 

Glycogen phosphoylase doesn't need ATP energy to attack the glycosidic bond and to phosphorylase glucose unit which is very advantage because we use use this reserve when cellular energy is low 

16

Difference in glycogen phosphorylase and alpha amylase

Both can cleave the glycosidic bond of glycogen chain. 

Glycogen phosphorylase does so by releasing phosphorylase without energy, while the alpha amylase attacks the amylase chain by hydrolysis so it using H2O to cleave the bond so it's a non phosphorylated glucose `

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Steps of Glycogenoylsis

1. Glycogen phoshoylase phosohorylsis breaking glycosidic bond yeirld Glucose 1-P. This stops 4 units away from a branching point. 

2. glucose 1-P isn't ready to be used so phosphoglucomutase attacks making it Glucose-6-P (which is a juntion point of either aerobis or anoerobic)

3. The liver has glucose 6-phosphatase which can produce glucose 

3B. In cells where this enzyme doesn't exist (or there isn't the translocase to move the GP6 into the ER) there is Type 1 glycogen storage. Periperal tissues use glycolysis producing either lactate or oxidation making CO2. 

4. Debranching enzyme- bifcuntioanl and 1st is transferase and moves a strand of 3 glocosyl residue from a 4 branch and transfer it to a free hydroxyl group of the same or adjacent glycogen molecle making the amylose chain longer 

18

Explain why we call the branching enzyme bifunctional

1. It is a transferase- transfer 3 of the 4 gluocse units to a non reducing end of an amylose chain (where the glycogen phosphorylase can handle it)

2. Glucoidase- cleaves the 1-6 glycosidc bond at the branching point making FREE GLUCOSE (remember that most glycogen degradation makes glucose phosphate but at a branch a point we make glucose-- this is good for the liver because it can immediately can be pushed out to the circulation; it also would need to be phosphorylated to enter the glycolitic pathway) 

19

What is McArdle syndrome

AKA type 5 is Complete loss of skeletal muscle phosphorylase (so the first step in glycogen degradation to make Glucose 1-P can't ever happen) . You can store glycogen but never use it. 

McArdle patient's suffer from painful cramps and cant preform strenuous exercise. They don't have the normal lactate build up due to exercise but the muscles are often damaged due to excess glycogen accumulation and low energy supply.

 

they can use creatine phosphate for quick 1 ATP energy 

CNS is normal because they use glucose and don't need gylcogen breakdown

Increae in myoglobin in the blood and urine-- when cells aren't getting enough energy they lyse and go in circulation 

 

 

 

20

What Hers disease

AKA Type VI is deficiency in LIvers phosphorylase isn't there, so you may have hypoglycemia during fasting. Normally glycogen is the first source to supply energy levels but other than hypoglycemia during fasting patients can be normal 

21

What is Pompe disease

TypeII glycogen disease is a lysosomal storage disease due to deficiency in Lysosomal 1-->4 glucosidase deficieiny. Glycogen accumulates in lysosome and it kills the cell. Can lead to cardiomegally is one of the first signs because so nothing can be degraded so build up

22

What is Cori's disease

Type III- the debrancher deficiency. So you can taper down the peripheral amylose chain until 4 glucose units away from branch point so you get some Glucose 6-p, but you're building this huge gylcogen molecule and you don't use it all. You can get Mild fasting hypoglycemia. 

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What is Type 1.Von Gierke's disease

two subtypes- but both have deficiency with the gluocse 6-phosphatase and affect only the liver and kidney (remember that the other cells go from Glucose 6-P to glycosolysis and odn't need to be broken down to glucose)

1A. Missing the G-6 phosphatase enzyme- this is the last step in the liver that removes the phosphate and makes free glucosen (most frequen)

 

1B. Missin translocase enzyme- enzyme that takes G6P from the cytosol to the ER for phosphatase attack through Glut 7 

 

1. serious hypoglycemia and the liver can't make any glucose! Very serious. 2. Enlarged renal, liver because glycogen is accumulating and sitting there.

3. Fatty liver- glucose 6-P is accumulating so it goes for fatty acid synthesis (some released and some stuck in liver). 

4.  Lactic acidosis,

5. can cause gout (through hyperuricema- push into the pentose pathway so more uric acid)

 

These patients should ALWAYS eat carbs/glucose. Even overnight through a tube 

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LIst some common myths of ED

1. Obestity is the most common ED-- false obestiy isn't an ED

2. Ppl are anorexia to look like models

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Common non-myths

1. ED carry the highest mortality rate of mental illness

2. Anorxia is closely related to birth order (youngest most likely)

3. Vegetarinism is an early marker of ED

 

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Peak populations in ED

Young adolescent girls and young womrn

Greater in white and asians

Two peak ages transitional 13-14 and 17-18 years

 

Only 5-10% in males

 

But remember ED are equal opportunity illness 

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Weight ranges in ED

Anorexia-nervosa- under weight (restrictive type and binge eating/purging)

 

Buliminia Nervosa- average weight

 

Binge eating- overwieght 

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Charateristics of anorecia

1. refusal to gain weight

2. Fear of gaining weight

3. Disturbance in the way ones body is experienced

4. Amerorhhea (removed from recent DSM)

 

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Characteristics of bulminia nervosa

1. Binge eating

2. COmpensatory purging

3. binge/purge at least 2x/week

4. Negatice self evaluation

5. Not during episodes of anerxia nervosa (cant be under weight) 

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Compensatory Behaviors in Bulimia nervosa

1. Self induced vomitting

2. MIsuse of laxatives, diuretics, enemas (metabolic stimulants)

3. Fasting

4. Extreme excerise

5. Diabetics failutre to take insulin 

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Why is ED an ethical dilemma

Its a emntal disorder with the highest mortality often due to medical complications, but weight loss is universal sign of illness

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Obstacles to early disorder

1. Patients fear

2. Patients hide it

3. Confidentiality hinder flow of info

4. Symptoms are hidden as other illnesses

5. Mistaken notion that patients dont want help

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Good questions to ask nin ED

1. How much do you want to weight

2. you or anyone concerns with your weight

3. Weight control methods

 

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Difference in body weight assestment in bulimina and anerxia

Anorexia- under eweight and can't gain or maintain

Bulimia- extreme fluctutions but usually in normal 

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five components of Detection to ED

1. History and physical

2. Interview friends and family

3. CMP

4. Self/family report instruemtns (EAT-26(

5. Follow up

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Two key components in treating

1. ED are more about weight than eating

2. Body weight is key to understanding and treating 

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treatment at RUsh discussed. 

phaes !- inpatient 

Phase II- outpaitent meal an weight monitored by family

Phase III- outpatient follow up

 

Let patietn develip own meal plan (you pick portions)

gradually increase meal quota

Hold patients accountable (circle failed meals in red, bed rest, naso-gastric when needed)

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Common myth about how patients see themselves

Most patients see themselves as their actual size but  affective (feel) and cognitive (think) they are too fat or fat 

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Which patients need the greatest calroies to gain weight

THINNIEST! Because SA/Weight= calories so they need the most to gain but maintaining declines.