Anytime (done perman) (m-10.4)

Question 1

PKU: pathway

Answer 1

Phenylalanine hydroxylase catalyzes PA -> tyrosine

no PAH
up PA

Question 2

Alkaptonuria pathway

Answer 2

Tyrosine -> homogentisic acid
HGA -> (HGA oxidase) -> fumarate

HGA oxidase def
HGA up

Question 3

Maple syrup urine disease pathway

Answer 3

BCCA breakdown cat by BC ketoacid dehydrogenase

X BCKD
up BCAA

Question 4

Which disease is most severe to brain (tremor, slurred speech, blurred vision)

Answer 4

Hyper ammonia

Question 5

hyperammonia genetic mec

Answer 5

ornithine trans carbamoylase def

Question 6

fructose metabolism rxn

Answer 6

f -> fk -> f 1 p -> aldolase -> glyceraldehyde 3 p -> glycolysis

Question 7

pentose phosphate rxn

Answer 7

G 6 p -> g 6 p dehydrogenase (rle) -> NADPH + ribose 5 p

Question 8

HDL: reverse transport of cholesterol from periphery to liver using what enzyme

Answer 8

LCAT

Question 9

how treat dys lipi demia?

Answer 9

give statin (blocks HMG coa reductase) -> reduce cholesterol

Question 10

list the major salivary glands and whether they are serous, mixed, or mucous

Answer 10

Parotid - serous
Sub mandibular - mixed
sub lingual - mucous

Question 11

histamine, creatine, and 5-HT are products of what rxn

mediated by what enz

Answer 11

rxn: decarboxylation
enz: pyridoxal phosphate

Question 12

indicator of liver damage

Answer 12

AST
ALT

asparate
alanine
aminotransferase

Question 13

congenital lactic acidosis symptom in urine

Answer 13

inc fumarate and AKG

Question 14

which disease is linked to non ketotic hypoglycemia

Answer 14

MCAD

Question 15

SCID is caused by what def

Answer 15

adenosine deaminase def

Question 16

flurouracil mec

Answer 16

inhibits thymidylate synthase (blocks dUMP to dTMP)

Question 17

ileal diverticulum is what

Answer 17

remnant of yolk stalk

read: pain similar to append.

Question 18

pain from appendicitis results from what

Answer 18

irritation of parietal peritoneum lining in the posterior ab wall

Question 19

which diseases cause RBC to lyse bc they lack a key energy source

Answer 19

pyruvate kinase def

hemolytic anemia

Question 20

describe mec of hemolytic anemia

Answer 20

def G 6 P dehydrogenase 
(cat G 6 P + NADP -> NADPH) 

no NADPH means no detox in RBC

anemia

Question 21

hemolytic anemia crises occur when

Answer 21

when exposed to

• antibiotic
• antimalarial
• antipyretic
• beans

Question 22

galactosemia mec

causes what symptoms

Answer 22

def of G 1 P uridyl transferase

baby liver disease and sepsis

Question 23

arsenic poisoning mec

Which disease is associated with Xanthoma?

Answer 23

inhibits pyruvate dehydrogenase

type 1 glycogen storage disease

Question 24

congenital lactic acidosis cause 2

Answer 24

def in pyruvate dehydrogenase
(normal: pyruvate -> ACA)
Pathway in diseased person: glucose -> pyruvate (low G, high pyruvate) -> but cannot use pyruvate -> lactate compensates (high lactate)

Question 25

two way flow occurs in hepatic vessel (be specific)

Answer 25

cystic duct

Question 26

draw out pyrimidine synthesis rxn

Answer 26

glutamine -> via carbamoyl phosphate synthase 2 (RLE) -> CP -> orotate (final added to PRPP) -> OMP -> UMP -> UDP -> UTP -> glutamine donates AA -> CTP

Question 27

foregut: sympathetic innervation mid hind

Answer 27

greater splanchnic -> celiac ganglia mid: lesser s -> sup mesenteric gang h: lumbar s -> IMG

Question 28

which organ starts off as two separate buds, but one swings around 180 and joins with the second bud to form the organ (on the dorsal side)

Answer 28

pancreas

Question 29

Type 1 glycogen storage disease caused by

Answer 29

def G 6 phosphatase norm: G6P -> glucose low glucose means get diabetes symptoms

Question 30

name the three drugs that are associated with THF/folate

Answer 30

methotrexate flurouracil solfonamide

Question 31

allopurinol is used to treat what disease

Answer 31

Gout

Question 32

Hartnup's disease mec

Answer 32

``` def tryptophan (resembles pellagra) (norm: try -> niacin) ```

Question 33

biosynthesis of non essential AA is accomplished via what type of rxn

Answer 33

trans animation

Question 34

Vitamin B6 is involved in what rxn (MAIN)

Answer 34

AA 1 + AKA 2 -> via B6/pyridoxal phosphate -> alpha keto acid 1 + AA 2

Question 35

nitrogen is transported to liver via what peripheral tissue uses what as its carrier how about muscle

Answer 35

carrier mol (created via transamination) Peri-glutamine mus-alanine

Question 36

which pathways use uridyltransferase

Answer 36

``` 1 glycogenesis (G6P -> G1P -> via UDT -> UDP G -> via G synthase -> glycogen) 2 galactose metabolism (Galactose 1 P -> via UDT -> glucose 1 P) ```

Question 37

which pathway uses citrate as its first step

Answer 37

fatty acid synthesis

Question 38

which pathway uses HMG coA synthase

Answer 38

ketogenesis

Question 39

the "tube" that is the intestine rotates around what thing (like an axis) final form is the twisted mass that is the intestine

Answer 39

superior mesenteric artery

Question 40

write out pyrimidine synthesis

Answer 40

glutamine -> carbamoyl phosphate synthase 2 -> carbamoyl phosphate -> orotate -> OMP -> UMP -> UDP -> UTP -> glutamine donates amino group -> CTP

Question 41

acini cells produce enzymes that are released in response to what chemical

Answer 41

CCK

Question 42

CCK is secreted by (what) cells (letter in alphabet)

Answer 42

I as in ice cream

Question 43

types of cells in exocrine pancreas | function

Answer 43

- ductal (secrete bicarbonate in response to secretin) | - centroacinar (just a ductal cell in center of acinus)

Question 44

three things in portal triad in liver: hepatic artery, bile duct, and portal vein which of these goes OUT of the liver

Answer 44

bile duct

Question 45

describe the VENOUS pathway thru liver and after it

Answer 45

portal vein goes into liver in liver, portal vein goes to central vein leaving the liver: CV -> hepatic vein -> IVC

Question 46

name all the things that can inhibit things in the ETC

Answer 46

aspirin - inhibits proton gradient across IMM rotenone - inh complex 1 cyanide, CO, sodium azide - inh complex 4 oligomycin - inh ATP synthase