PHAII

Question 1

Features of PHAII

Answer 1

Hyperchloremia
Hyperkalemia
Hypertension
Metabolic Acidosis

Question 2

Hyperkalemia range

Answer 2

K+ above 5.5

Question 3

Hyperchloremia range

Answer 3

Cl- above 110

Question 4

Hypertension

Answer 4

BP above 140/90

Question 5

Metabolic acidosis

Answer 5

pH below 7.35

Question 6

Two mutations causing PHAII

Answer 6

WNK1 gain of function (autosomal dominant)

WNK4 Loss of function (autosomal recessive)

Question 7

What condition is PHAII clinically inverse to

Answer 7

Gitelman’s syndrome

Question 8

E2s

Answer 8

Ubiquitin conjugating enzymes

Question 9

E1s

Answer 9

Ubiquitin Activating enzymes

Question 10

E3s

Answer 10

Ubiquitin Ligases

Question 11

Ub Activation

Answer 11

E1 (ATP dependent) binds Ub (with ATP bound)

ATP–>AMP+2Pi

Question 12

Ub Conjugation

Answer 12

E2 catalyses Ub trasnfer from E1 to cysteine active site of E2 via trans(thio)esterification

Question 13

Ub Ligation

Answer 13

E3 (with substrate bound) binds E2
Transubiquitination - Ub transferred to substrate
E2 left with just SH as it started