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1

This lecture sucks hairy fucking balls

FML

2

what would an absence of cortisol cause?

death by hypoglycemia

3

what is the purpose of the biotin cycle?

carboxylase creation and get pruvate out of mito matrix to cytosol

4

what condition does a biotin deficiency lead to?

lactic acidosis

5

what symptoms does lactic acidosis lead to?

rashes, brittle hair, etc.

6

how do you measure biotin?

urine

7

how do you measure biotindiase?

biocytin in urine

8

pepck def is inherited how?

autosomal recessive

9

what does PEPCK do?

oxaloacetate to PEP

10

what symptoms does an individual with PEPCK def?

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

11

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

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

12

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

egg whites

13

Von Gierke Disease is called what?

glycogen storage dz 1a

14

what enyzme is def in VG dz?

G6P-ase

15

what are symptoms of VG dz?

hypoglycemia, lactic acidosis, hepatomegaly and *Retarded Growth*

16

GSD 1b is due to what?

def. in transporter of G6P from cytosol to ER

17

symptoms of GSD1b?

hypoglycemia

18

how does someone recover from GSD1b?

liver transplant

19

what is the primer for glycogen synthesis?

glycogenin

20

what is the rate-limiting step in glycogenolysis?

glycogen phosphorylase creating G1P

21

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

Hers dz; GSD6

22

Dz that results from glycogen phosphorylase in skeletal m.?

mcardle dz; GSD 5

23

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

cori dz; GSD3

24

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

glucosyl 4:6 transferase absent; GSD 4

25

what does GSD4 result in in cells?

very long, unbranched glycogen?

26

what does pompe dz result from? other name?

acid maltase def; GSD 2