Theme 2

Question 1

Link reaction

• where
• enzyme
• reaction type

Answer 1

Matrix
Pyruvate dehydrogenase
Oxidative decarboxylation

Question 2

Committed step enzyme

Answer 2

PFK-1

Question 3

SLP enzymes

Answer 3

Hk and PFK-1

Question 4

TCA products

Answer 4

3NADH, 1 FADH2, GTP, 2CO2

Question 5

Inner membrane impermeable to

Answer 5

NADH

Question 6

Coenzymes [5]

Answer 6

NAD, Biotin, TPP, CoA, Lipoic acid

Question 7

Riboflavin

Answer 7

B2

FAD

Question 8

Thiamine

Answer 8

B1

TPP

Question 9

Niacin

Answer 9

NADP

Question 10

Pantothenate

Answer 10

B5

CoA

Question 11

FAD

Answer 11

Riboflavin

B2

Question 12

CoA

Answer 12

Pantothenate

B5

Question 13

TPP

Answer 13

Thiamine

B1

Question 14

NADP

Answer 14

Niacin

Question 15

ROS [5]

Answer 15

Superoxide anion 
Hydroxyl radical 
Hydrogen peroxide 
Peroxide ion 
Hypochlorous acid

Question 16

Mt disease overview

Answer 16

150 diff types
16.5kb
5-10 copies
900 diff mt proteins

13 resp chain proteins, 2 rRNA and 22 tRNA

Question 17

Mt myopathies

Answer 17

1. Defects of mitochondrial transport systems
Carnitine palmitoyltransferase (CPT I and II) deficiencies

2. Defects of substrate utilisation
   Pyruvate dehydrogenase complex (PDC) deficiency
   Fatty acid oxidation defects
3. Defects of TCA cycle
   Fumarase deficiency OR - ketoglutarate dehydrogenase deficiency
4. Defects of OXPHOS coupling
   Luft’s syndrome
5. Defects of oxidative phosphorylation
   Complexes I / II / III / IV / V deficiencies combined defects of respiratory chain components

Question 18

In LHON, electron transport from what is insufficient

Answer 18

Succinate

Question 19

MERF

Answer 19

TK, TL1, TH, TF
Oxidative phosphorylation
Point mutation - tRNA for tyrosine

Question 20

MELAS

Answer 20

ND5

TH, TL1, TF

Question 21

KSS

Answer 21

5kb deletion
short stature, multiple endocrinopathies
Lactic acidosis, heart conduction defect and CSF protein content

Question 22

Normal glucose concentration

Answer 22

2.5-8mM

Question 23

Insulin actions [4]

Answer 23

Increase liver enzymes - synthesise glycogen
Increase adipocyte enzymes - synthesise TG

Inhibit lipolysis
Function as growth factors

Question 24

Phosphate pentose pathway

Answer 24

Cystolic pathway

Branch from G6P

Question 25

Increase G6P inhibits

Answer 25

Hexokinase -> prevent glucose phosphorylation

Question 26

Glucagon and adrenaline

Answer 26

Activate glycogen breakdown and inhibit CAMP PK

Question 27

Irreversible steps

Answer 27

G6Pase, F16BPase, pyruvate carboxylase and PEP carboxykinase

Question 28

Phosphorylation effect on PFK2 and F26BPase

Answer 28

Inhibit PFK2 and stim F26BPase

Question 29

Increase FA = Increase A CoA

Answer 29

Activate pyruvate carboxylase and inhibit pyruvate dehydrogenase

Question 30

Increase Glucagon

Answer 30

Inhibit PFK2 and slim F26BPase

Question 31

Decrease F26BP

Answer 31

Decrease PFK1 and relieve F16Pase inhibition

Question 32

Catecholamine actions [3]

Answer 32

Stim glucogen breakdown
BV compressed
Breakdown phosphocreatine

Question 33

Type 1 Diabetes causes

Answer 33

Autoimmune B cell destruction

`viral infection

Question 34

Type II Diabetes [3]

Answer 34

Impaired insulin secretion (amyloid deposit = less B cell mass)

Increased peripheral resistance (increase FA)

Increased hepatic glucose output

Question 35

Type II diabetes drugs

Answer 35

Sulphonylureas (Gliclazide)
Metformin
Thiazolidinedione

Question 36

Sulphonylureas

Answer 36

Glucose depolarise membrane & ATP-dependent K+ channel closing = Ca2+ influx = insulin secretion

Question 37

Metformin

Answer 37

No hypoglycaemia & suppress appetite

Increased insulin insensitivity

Question 38

Thiazolidinedione

Answer 38

Reduce hepatic glucose output
Increase muscle uptake
Enhance insulin effectiveness
Bind to nuclear receptor (peroxisome proliferator activated receptor)

Question 39

Hypoglycaemia

Answer 39

Below 4mM (2.5mM = unconscious)

Hypernatraemia
Hypovolaemia
Alcohol Pathologies - Adrenal Insufficiency