Biochem- Aa Metabolism

Front 1

in order to make protein you need ALL amino acids T/F

Back 1

T

Front 2

what are essential amino acids

Back 2

aa we can't synthesize

Front 3

negative nitrogen balance

Back 3

excreting more nitrogen than taking in (e.g., Kwashikor's disease)

Front 4

positive nitrogen balance

Back 4

taking in more nitrogen than excreting

Front 5

this amino acid is essential only in growth phases

Back 5

histadine

Front 6

name the tca cycle intermediate that produces:
1)FA
2)AA synthesis (neurotransmitters)
3)heme synthesis
4)gluconeogenesis
5)amino acids

Back 6

1)citrate
2)alpha ketogluterate
3)succinyl coA
4)malate
5)oxaloacetate

Front 7

glycogenic

Back 7

can become glucose or glycogen

Front 8

ketogenic

Back 8

can become ketone bodies

Front 9

leucine is the only strictly ___________ amino acid.

Back 9

ketogenic. All others are glycogenic or both

Front 10

Alanine is converted to pyruvate via what enzyme?
How is this reaction classified?

Back 10

ALANINE TRANSAMINASE ENZYME(serum glutamate pyruvate transaminase)
Transaminase rxn

Front 11

Aspartate is converted to oxaloacetate by using what?
How is this reaction classified?

Back 11

alpha ketogluterate
glutamate
Transaminase rxn

Front 12

Glutamate is converted to glutamine via what enzyme

The reverse happens with what enzyme?

Back 12

glutamine synthase enzyme

glutaminase

Front 13

how do you diagnosse cystinurea?

Back 13

assay urine for lycine, ornithine, arginine

Front 14

______ bonds in homocystein molecules can lead to DVT, thrombosis, & hypercoagulation in homocystein urea

Back 14

disulfide

Front 15

what enzyme is involved in homosystein urea

Back 15

cystathione synthase

Front 16

cystathione synthase is a _____ containing enzyme, meaning _____ vitamen deficiencis may effect it.

Back 16

PLP, vB6

Front 17

describe enzyme and cofactors involved in cystathionin urea

Back 17

Cystathionin urea involves the cystathion lyase enzyme it is a PLP containing enzyme (B6)

Front 18

What enzymes are fed into the proprionic acid pathway

Back 18

Valine
or
Methionine
Isoleucine
Threonine
(VoMIT)

Front 19

in PAP, Homosystein comes from _______

Back 19

methionine (homosystein + methyl)

Front 20

Maple Syrup Urine Disease
1)enzyme
2)AA metabolism affected

Back 20

1)Branch chain alpha ketoacid dehydrogenase
2)leucine, isoleucine, valine

Front 21

PKU
1)enzyme
2)problem
3)sweetener to avoid

Back 21

1)phenalalanine hydroxylase
2)phenyl ketones build up in blood stream
3) aspartame

Front 22

phenyl alanine is transaminated to produce which phenyl ketones in PKU?

Back 22

phenyllactate & phenyl pyruvate

Front 23

Alkaptonuria
1)enzyme
2)AA metabolism involved
3)clinical signs & symptoms

Back 23

1)homogentisate dioxygenase
2)phenylalanine & tyrosine
3)Dark urine, ocronosis (darkening of jts), & arthritis

Front 24

Alkaptonurea
1)enzyme
2)pathway
2)clinical signs

Back 24

1)homogentisate oxidase
2)AA metabolism
3)Dark Urine, Ochronosis:Darkening of the joints & Arthritis

Front 25

nongenetic causes of homocysteine urea

Back 25

deficiency of B6 (folate) & B12

Front 26

enzymes that use B12 as cofactor (give pathway as well)

Back 26

1)Homocysteine Methyltransferase (methionine synthase)
2)Methmalonyl-CoA Mutase (proponyl CoA pathway)

Front 27

what is build-up of methmalonyl CoA is called and what is diagnostic for

Back 27

methmalonyl urea
B12 deficiency

Front 28

Dihydrofolate reductase-

Back 28

converts Folate(F)to Dihydrofolate(FH2)to etrahydrofolate(FH4) by methyl transferase. Important in making DNA from RNA

Front 29

thymadylate synthase

Back 29

takes dUMP to dTMP, N5,N10 methylene FH4 is carrier of methyl group
-inhibited by 5-FU (chemotherapy)

Front 30

dihydrofolate reductase

Back 30

takes FH2 to FH4
-inhibited by methotrexate (chemotherapy)

Front 31

sx of methotrexate similar to _______

Back 31

folate

Front 32

SAM-- what does it do and give example

Back 32

used directly for methyllations
(e.g., Norepinephrine -- epinephrine;Nucleotides -- methalinated Rxns)

Front 33

Homocysteine Methyltransferase (methionine synthase)

Back 33

key enzyme in methylation cycle. Converts homocystein to methionine. Uses B12 as a cofactor and ALSO B6 which interacts w/ B12

Front 34

Maple syrup Urine disease
1)enzyme
2)clinical symptoms

Back 34

1)branched chain keto acid dihydrogenase
2)urine has oder of maple syrup,mental retardation,abnormal mm tone,ketosis, coma, death

Front 35

PKU
1)enzyme
2)clinical symptoms

Back 35

1)phenylalenine hydroxylase
2)mental retardation, microcephaly, diet important, musty odor,

Front 36

causes of homocystein urea

Back 36

Vit B12 blockage
Vit B6
Genetic disorder

Front 37

differential diagnosis of homocystein urea

Back 37

B12 deficiency you will also see associated methomalonic acid urea.
If not B12 give B6 if doesn't recover then genetic

Front 38

How does B12 deficiency induce B6 deficiency

Back 38

Folate trap. We need B12 as a cofactor for homocystein methyl transferase. HMT is necessary to recylce folate from it's inactivated reduced methylated form (methylated THF) to it's active form THF.

Front 39

megablastic anemia check for

Back 39

homoceistein urea

Front 40

Megablastic anemia due to folate deficiency:

Back 40

Homocystinurea
Developes 3-4 months
Risk factors for deficiency:
-pregnancy
-alcoholism
-severe malnutrition

Front 41

Megaloblastic anemia due to Vit B12 (Cobalamin) deficiency

Back 41

Progressive peripheral neuropathy*
Homocystinemia w/ risk for cardiovascular disease
Methylmalonic aciduria*
Deficiency develops in years
Risk factors:
-Pernicious anemia
-gastric resection
-chronic pancreatitis
-severe malnutrition
-vegan
-infection w/ D. latum

Front 42

Pernicious anemia

Back 42

Subset of Megaloblastic anemia. Due to lack of intrinsic factor inhibiting B12. Will have w/ vit B12 deficiency.