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MCB 2 Block 3 > Amino acid Degrade and Synth > Flashcards

Amino acid Degrade and Synth Flashcards

1
Q

Tetrahydrofolate

A 
TH4/BH4 = Vit B9, used for methylation
Enzyme = dihydrofolate reductase, rqrs NADPH x2
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2
Q

SAM general

A

S-Adenosyl Methionine used for Methylation

Synth from methionine and ATP

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3
Q

SAM synth pathway drawn

A

On paper

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4
Q

SAM precursor to Final

A 
NE to Epi
Guandinoacetate to Creatine
Nucleotides to meth-nucs
Phosphatidylethanolamine to Phosphatidyl choline
AcetylSerotonin to Melatonin
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5
Q

BH4

A

Tetrahydrobioptrin used for hydrolation, B9

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6
Q

Vitamin C

A

Ascorbate for hydroxylation

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7
Q

Alanine

A

Comes from pyruvate through transamination

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8
Q

Arginine

A

Ultimately from glu via urea cycle (glu = ornithine = Arg)

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9
Q

Aspartate

A

Comes from OAA through transamination

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10
Q

Asparagine

A

Comes from Asp via Asparagine sythetase
Requires ATP and NH3 source
Breakdown back to asp via asparaginase

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11
Q

Cysteine

A

Comes from Met (supps Sulfur) and Ser (supps C and N)

Met to SAM to SAH to homocysteine
SAM to SAH via methyl donation to X
Homocysteine to Met via THF and B12
Homocysteine and Ser to cystathione to Cys and alphaketobutyrate (rqrs PLP x2, alpha ketobutyrate to propionyl COA to Succinyl COA)

Cys to Taurine via cysteine sulfinate decarboxylase (No codon for taurine so no protein incorp, rapidly metab)

Sulfate of Cys to PAPs, via sulfotransferases rqrs 3 ATP equivs

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12
Q

Glutamate

A

Comes from alpha-ketoglutarate (glucose = C source) through transamination or deamination

Glu to Pro, Gln, Arg
His, Pro, Gln, Arg to Glu

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13
Q

Glutamine

A

Comes from Glu via glutamine synthetase

rqrs NH3

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14
Q

Glycine synth

A

Comes from Ser and Thr
Gly = Ser via FH4
Thr = Gly via PLP

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15
Q

Glycine Degrade 1

A

Gly to CO2 and NH2
Rqrs THF and NAD
Enz = glycine cleavage enzyme

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16
Q

Defect in glycine cleavage enzyme

A

hyperglycinemia, fatal

mental retard and hiccups, excess gly (NTrans) = lots of inhib

17
Q

Glycine Degrade 2 and defect

A

Gly to glyoxylate
Defect = primary oxaluria type 1
excess glyoxylate leading to kidney stones

18
Q

Histidine

A

Essential
His to FIGLU
enz = histidase; rqrs THF

19
Q

Isoleucine

A

Essential
Ile to propionyl CoA to Succinyl Coa
Enz=BCKAD
Rqrs TLCFN (Tpp, lipoc acid, CO2, FAD, NAD)

20
Q

BCKAD def

A

Maple syrup urine disease
Severe mental retard
Tx = B1 (thiamine) which inhibits BCAA uptake

21
Q

Leucine

A

Essential
Leu to acetylaceto CoA to Acetyl CoA
Enz = BCKAD; rqrs TLCFN

22
Q

Lysine

A

Essential
Lys to acetylaceto CoA to Acetyl CoA
Lys to Carnitine (rqrs 3 SAMs)

23
Q

Methionine

A

Essential

Met to SAM to SAH to homocysteine (previous)

24
Q

Folate Trap

A

When THF cant be recycled due to B12 def

25
Q

Homocystinuria

A

Defect in Cystathione Synthase leading to buildup of homocysteine = Met and homocystine buildup
Looks similar to Marfans but joints are tight with Funnel Chest

26
Q

Cystinosis

A

Defect in lysosomal transport, cysteine dimer to cystine

27
Q

Cystinuria

A

Defect in AA transport in kidneys forming cystine kidney stones

28
Q

Cystithionuria

A

Defects in Cystathionase leading to cystathione build up and is benign

29
Q

Phenyalanine

A

essential
Phe to Tyr
Enz = phenylalanine hydroxylase (PAH); rqrs BH4

30
Q

PKU

A 
Classic = def of PAH
Malignant = def of BH4 (also Tyr and Trp metab)
Maternal = high mom Phe is teratogenic for fetus leading to PKU in baby

Unable to degrade Phe so diverted to other routes, conversion to phenylacetate and phenyllactate hurts brain

31
Q

Proline

A

Comes from cyclization of Glu

32
Q

Serine

A

From glycolysis; ser = gly (to gly via serine hydroxymethyl transferase; rqrs TH4)

Ser minus NH3 to pyruvate (rqrs Serine dehydratase)
Ser plus homocysteine to Cys

33
Q

Threonine

A

essential
Thr to gly via PLP
Thr minus NH3 to pyruvate and alpha ketobutyrate (via Threonine dehydratase)

34
Q

Tryptophan

A

essential
Trp to niacin and acetylaceto CoA
Endogenous source of niacin
Some excretion products (kyurinate, xanthurenate)

35
Q

Tyrosine

A

Comes from Phe via phenyl alanine hydroxylase; rqrs O2 and BH4
Tyr to hydroxyphenylpyruvate (PLP)
Hydroxyphenylpyruvate to homogentisate (via homogentisate oxidase; rqrs Vit C)
Homogentisate to fumurate and acetoacetate

36
Q

Alkaptonuria

A

Benign
Def = homogentisate oxidase, urine black with air contact
Leads to ochronosis (dark pigment in CT)

37
Q

Tyrosinemia

A

type 1: last rxn of tyrosine, cabbage odor

type 2: PLP step of Tyrosine

38
Q

Valine

A

essential
Val to Propionyl CoA to Succinyl CoA
Enz = BCKAD; rqrs TLCFN

MCB 2 Block 3 (13 decks)

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