Liver Function II - Seidler

Question 1

This lecture sucks hairy fucking balls

Answer 1

FML

Question 2

what would an absence of cortisol cause?

Answer 2

death by hypoglycemia

Question 3

what is the purpose of the biotin cycle?

Answer 3

carboxylase creation and get pruvate out of mito matrix to cytosol

Question 4

what condition does a biotin deficiency lead to?

Answer 4

lactic acidosis

Question 5

what symptoms does lactic acidosis lead to?

Answer 5

rashes, brittle hair, etc.

Question 6

how do you measure biotin?

Answer 6

urine

Question 7

how do you measure biotindiase?

Answer 7

biocytin in urine

Question 8

pepck def is inherited how?

Answer 8

autosomal recessive

Question 9

what does PEPCK do?

Answer 9

oxaloacetate to PEP

Question 10

what symptoms does an individual with PEPCK def?

Answer 10

hepatomegaly and hypoglycemia, acidemia, loss of m. tone, failure to thrive

Question 11

What does F1,6 BP deficency cause? and when?

Answer 11

lactic acidosis, hypoglycemia, hyperventilation, ketosis, apnea; early childhood

Question 12

what should you not eat if you have a biotin def?

Answer 12

egg whites

Question 13

Von Gierke Disease is called what?

Answer 13

glycogen storage dz 1a

Question 14

what enyzme is def in VG dz?

Answer 14

G6P-ase

Question 15

what are symptoms of VG dz?

Answer 15

hypoglycemia, lactic acidosis, hepatomegaly and Retarded Growth

Question 16

GSD 1b is due to what?

Answer 16

def. in transporter of G6P from cytosol to ER

Question 17

symptoms of GSD1b?

Answer 17

hypoglycemia

Question 18

how does someone recover from GSD1b?

Answer 18

liver transplant

Question 19

what is the primer for glycogen synthesis?

Answer 19

glycogenin

Question 20

what is the rate-limiting step in glycogenolysis?

Answer 20

glycogen phosphorylase creating G1P

Question 21

if you have a glycogen phosphorylase def in the liver you have what dz?

Answer 21

Hers dz; GSD6

Question 22

Dz that results from glycogen phosphorylase in skeletal m.?

Answer 22

mcardle dz; GSD 5

Question 23

what dz results due to def in alpha 1,6 glucosidase?

Answer 23

cori dz; GSD3

Question 24

Andersen Disease results due to def of what? other name?

Answer 24

glucosyl 4:6 transferase absent; GSD 4

Question 25

what does GSD4 result in in cells?

Answer 25

very long, unbranched glycogen?

Question 26

what does pompe dz result from? other name?

Answer 26

acid maltase def; GSD 2