Turco 3

Question 1

Glc gives up how many total pairs of electron

Answer 1

12 pairs
1 in glycolysis to pyruvate
5 from pyr to CO2

Question 2

Step of glycolysis giving up electrons

Answer 2

Glyceraldehyde 3P —-> 1,3 BPGT

Question 3

Pyruvate dehydrogenase complex

Answer 3

Takes pyruvate to acetyl CoA
Located in Mitochondria
Releases pair of electrons

Question 4

Isocitrate dehydrogenase

Answer 4

Takes isocitrate to alpha ketoglutarate

Creates NADH from NAD

Question 5

Regulation of isocitrate dehydrogenase

Answer 5

Turned off by high levels of ATP and NADH…this will increase concentration of citrate that then inhibits PFK1 of glycolysis in EXTRAhepatic cells

Question 6

Creatine and ATP

Answer 6

ATP cannot be stored in cells so phosphate added to creatine for storage

Question 7

Creatine kinase isoforms

Answer 7

Skeletal muscle - MM
Brain - BB
HEart - MM and MB isoform

Question 8

Importance of MB isoform

Answer 8

Indicative of heart attack…creatine kinase will spill out into the bloodstream and be present for about 24 hours

Question 9

Creatine metabolism

Answer 9

Creatine converted to creatine phosphate using ATP

Creatine phosphate turns into creatinine nonenzymatically with release phosphate

Question 10

In very short burts?

Answer 10

ATP levels stay relatviely the same (enzyme that takes 2 ADP to ATP+AMP)
Creatine-P will decrease dramatically

Also lactic acid

Question 11

Intermediate energy expenditure

Answer 11

Mix of fatty acid and aerobic

Question 12

Long term energy expenditure

Answer 12

Glycogen is gone so increased fatty acid metabolism

Question 13

TG metabolism in adipose

Answer 13

HSL activated by epinephrine and cortisol, decreased by insulin
Phosphorylated enzyme takes TGs to Glycerol and FAs

Question 14

TG metabolism in liver

Answer 14

Glycerol moved into liver and taken to glucose via gluconeogenesis (activated by glucagon and cortisol)
FA moved into liver and unergo B-oxidation to AcCoA
AcCoA either goes to krebs or undergoes ketogenesis to form ketone bodies

Question 15

When will TG metabolism start

Answer 15

90 minutes after last carb

Question 16

No receptor for glucagon in

Answer 16

Adipose tissue

Question 17

Insulin, epinephrine, and cortisol mech of HSL

Answer 17

Insulin - dephosphorylate
Epi - phosphorylate
Cortisol - genomic level

Question 18

Why is B-oxidation important?

Answer 18

Creates energy necessary to drive gluconeogenesis

Question 19

Why would defect in B-oxidation cause SIDS?

Answer 19

Can’t drive gluconeogenesis so hypoglycemia

Question 20

Ketogenesis, B oxidation and gluconeogensis locations

Answer 20

Keto and gluco in liver only

B-oxidation can be in other cells

Question 21

Atkins diet rationale

Answer 21

Keep insulin low by decreasing carbs in diet…this means HSL always active

Question 22

Problem with atkins diet

Answer 22

Used muscle proteins from glucogenic amino acids to make appropriate glucose

Question 23

Other probs with atkins

Answer 23

Excessive N secretion (kidney)

Body is in long term ketosis

Question 24

FA transport into matrix for oxidation

Answer 24

CoA added to FA when bringing in through outer membrane…CoA then removed and FA transferred to carnitine by CAT…moved through inner membrane…CAT 2 removes carnitine and makes FA-CoA again

Question 25

FA-CoA in the matrix

Answer 25

Processed by LCAD, MCAD, or SCAD and forms FADH2...also form NADH...2 carbons removed in form of Ac-CoA This is repeated until down to four and then split into 2 Ac-CoA

Question 26

KB metabolism

Answer 26

2 acetyl CoA get to acetoacetate Acetoacetate forms B-hydroxybutyrate with NADH OR decarboxylated to form acetone Acetoacetate and B-hydroxybutyrate moves out of blood

Question 27

How are ketone bodies used

Answer 27

Broken down into 2 acetyl CoA inside extrahepatic cells

Question 28

Hallmarks of ketosis

Answer 28

INcreased KBs leads to ketonemia (bad bc pH will drop) Increased KBs in urine leads to ketonuria....bad because dehydration Acetone breath (fruity odor)

Question 29

Myopathic canitine deficiency presentation

Answer 29

Elevated muscles TGs and reduced muscle carnintine onyl...muscle weakness severe during exercise

Question 30

Myopathic carnitine deficiency pathology

Answer 30

Could be defect of CAT enzymes, transporters, or error in carnitine creation FAs will stay in the muscle cell and TGs cannot exit the muscle Epinephrine activates adipose tissue and triggers HSL...TGs breakdown and enter cells...FA cannot go into matrix to be burned

Question 31

MCAD def presentation

Answer 31

Hypoglycemia and hypoketonemia Dicarboxylic acidemia (from omega oxidation) C8/C10 acylcarnitines in blood

Question 32

MCAD def pathology

Answer 32

Hypoglycemia because without MCAD, cannot power gluconeogenesis...normally hypoglycemia means hyperketonemia

Question 33

Propionate metabolism

Answer 33

5 carbon fragment split to acetyl CoA and proprionyl CoA (3 carbons) Propionyl CoA carboxylase takes propionyl CoA to methylmalonyl CoA using (ATP, biotin, CO2) Methylmalonyl CoA mutase takes methylmalonyl CoA to succinyl CoA using coenzyme form of Vit B12

Question 34

Propionyl CoA also made from

Answer 34

Val, Met, Ile, and Thr

Question 35

If priopionyl CoA carboxylase borken

Answer 35

Propionic acid in the urine and blood

Question 36

Defect in methylmalonyl CoA mutase

Answer 36

Causes methylmalonic acidemia and gives rise to peripheral neuropathy as integrated into myelin sheaths

Question 37

Causes of methylmalonyl CoA mutase def

Answer 37

``` B12 def IF def (binds to B12 to absorb) Mutase defect Defect in coenzyme form of B12 (can't convert ```