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42 Cards in this Set

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in order to make protein you need ALL amino acids T/F
T
what are essential amino acids
aa we can't synthesize
negative nitrogen balance
excreting more nitrogen than taking in (e.g., Kwashikor's disease)
positive nitrogen balance
taking in more nitrogen than excreting
this amino acid is essential only in growth phases
histadine
name the tca cycle intermediate that produces:
1)FA
2)AA synthesis (neurotransmitters)
3)heme synthesis
4)gluconeogenesis
5)amino acids
1)citrate
2)alpha ketogluterate
3)succinyl coA
4)malate
5)oxaloacetate
glycogenic
can become glucose or glycogen
ketogenic
can become ketone bodies
leucine is the only strictly ___________ amino acid.
ketogenic. All others are glycogenic or both
Alanine is converted to pyruvate via what enzyme?
How is this reaction classified?
ALANINE TRANSAMINASE ENZYME(serum glutamate pyruvate transaminase)
Transaminase rxn
Aspartate is converted to oxaloacetate by using what?
How is this reaction classified?
alpha ketogluterate
glutamate
Transaminase rxn
Glutamate is converted to glutamine via what enzyme

The reverse happens with what enzyme?
glutamine synthase enzyme

glutaminase
how do you diagnosse cystinurea?
assay urine for lycine, ornithine, arginine
______ bonds in homocystein molecules can lead to DVT, thrombosis, & hypercoagulation in homocystein urea
disulfide
what enzyme is involved in homosystein urea
cystathione synthase
cystathione synthase is a _____ containing enzyme, meaning _____ vitamen deficiencis may effect it.
PLP, vB6
describe enzyme and cofactors involved in cystathionin urea
Cystathionin urea involves the cystathion lyase enzyme it is a PLP containing enzyme (B6)
What enzymes are fed into the proprionic acid pathway
Valine
or
Methionine
Isoleucine
Threonine
(VoMIT)
in PAP, Homosystein comes from _______
methionine (homosystein + methyl)
Maple Syrup Urine Disease
1)enzyme
2)AA metabolism affected
1)Branch chain alpha ketoacid dehydrogenase
2)leucine, isoleucine, valine
PKU
1)enzyme
2)problem
3)sweetener to avoid
1)phenalalanine hydroxylase
2)phenyl ketones build up in blood stream
3) aspartame
phenyl alanine is transaminated to produce which phenyl ketones in PKU?
phenyllactate & phenyl pyruvate
Alkaptonuria
1)enzyme
2)AA metabolism involved
3)clinical signs & symptoms
1)homogentisate dioxygenase
2)phenylalanine & tyrosine
3)Dark urine, ocronosis (darkening of jts), & arthritis
Alkaptonurea
1)enzyme
2)pathway
2)clinical signs
1)homogentisate oxidase
2)AA metabolism
3)Dark Urine, Ochronosis:Darkening of the joints & Arthritis
nongenetic causes of homocysteine urea
deficiency of B6 (folate) & B12
enzymes that use B12 as cofactor (give pathway as well)
1)Homocysteine Methyltransferase (methionine synthase)
2)Methmalonyl-CoA Mutase (proponyl CoA pathway)
what is build-up of methmalonyl CoA is called and what is diagnostic for
methmalonyl urea
B12 deficiency
Dihydrofolate reductase-
converts Folate(F)to Dihydrofolate(FH2)to etrahydrofolate(FH4) by methyl transferase. Important in making DNA from RNA
thymadylate synthase
takes dUMP to dTMP, N5,N10 methylene FH4 is carrier of methyl group
-inhibited by 5-FU (chemotherapy)
dihydrofolate reductase
takes FH2 to FH4
-inhibited by methotrexate (chemotherapy)
sx of methotrexate similar to _______
folate
SAM-- what does it do and give example
used directly for methyllations
(e.g., Norepinephrine -- epinephrine;Nucleotides -- methalinated Rxns)
Homocysteine Methyltransferase (methionine synthase)
key enzyme in methylation cycle. Converts homocystein to methionine. Uses B12 as a cofactor and ALSO B6 which interacts w/ B12
Maple syrup Urine disease
1)enzyme
2)clinical symptoms
1)branched chain keto acid dihydrogenase
2)urine has oder of maple syrup,mental retardation,abnormal mm tone,ketosis, coma, death
PKU
1)enzyme
2)clinical symptoms
1)phenylalenine hydroxylase
2)mental retardation, microcephaly, diet important, musty odor,
causes of homocystein urea
Vit B12 blockage
Vit B6
Genetic disorder
differential diagnosis of homocystein urea
B12 deficiency you will also see associated methomalonic acid urea.
If not B12 give B6 if doesn't recover then genetic
How does B12 deficiency induce B6 deficiency
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.
megablastic anemia check for
homoceistein urea
Megablastic anemia due to folate deficiency:
Homocystinurea
Developes 3-4 months
Risk factors for deficiency:
-pregnancy
-alcoholism
-severe malnutrition
Megaloblastic anemia due to Vit B12 (Cobalamin) deficiency
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
Pernicious anemia
Subset of Megaloblastic anemia. Due to lack of intrinsic factor inhibiting B12. Will have w/ vit B12 deficiency.