1) Characteristics of Phenylketonuria (PKU)
- Alpha KG defect
- Up regulation go phenylalanine hydroxylase
- Abundance of dihydropteridine reductase
Correct answer: Deficiency of phenylalanine hydroxylase
2) Alcaptonuria
- Clear urine
- Homogenistate oxidase hyperactivity
Correct answer: Homogenistate oxidase deficiency
3) Not part of a treatment for PKU
- Controlled low phenylalanine diet
- Oral tetrahydrobiopterin (THB)
- Long term
Correct answer: High phenylalanine diet
4) Creatine
- Formed ezymatically, entirely excreted in urine
- Formed nonezymatically, not excreted in urine
Correct answer: Formed nonezymatically, entirely excreted in urine
5) Serotonin synthesis
- Serine->serotonin
- Melatonin->serotonin
Correct answer: Trp->serotonin
6) Tyrosinemia type 2
- Liver, kidney, peripheral nerve defects. Deficiency of Fumarylacetoacetate hydrolase
Correct answer: Eye, skin defects. Deficiency of tyrosine amino acid transferase
7) Coenzyme A synthesis
- Phosphopantothenate + Serine-> Anp + CoA-SH
- Tryptophan + Cystiene -> Anp + CoA-SH
- Phosphopantothenate + SAM -> Anp + CoA-SH
Correct answer: Phosphopantothenate + Cystiene -> Anp + CoA-SH
8) Pyriminde biosynthesis requires
- Glycine, Glutamine, Aspartate
- Serine, Aspartate
Correct answer: Aspartate, Glutamine
9) Polyamines
- Putrescine , spermidine, ornithine. Derived from spermine and methionine
- Putrescine , spermidine. Derived from ornithine and methionine
- Putrescence, spermidine, spermine. Derived from ornithine and methionine
Correct answer: Putrescine, spermidine, spermine. Derived from ornithine and methionine
10) NO synthesis
- Arginine-> Citrulline-> NO
- Serine-> Citrulline + NO
Correct answer: Arginine-> Citrulline + NO
11) SAM synthesis
- Serine, Methionine and ATP
- Tryptophan, NAD
- Succinate, co2, glycine
Correct answer: Methionine and ATP
12) Classical albinism caused by
- Aromatic acid decarboxylase deficiency
- Tyrosinase overabundance
Correct answer: Tyrosinase deficiency
13) E-n-trimethyllysine conversion to
- SAM
- NAD
- Tryptophan
Correct answer: Carnitine
14) Catecholamine synthesis
- Phe->Dopa->Dopamine->NE->Epi
- Tyr->Dopamine->Dopa->NE->Epi
- Tyr->Dopa->Dopamine->Epi->NE
Correct answer: Tyr->Dopa->Dopamine->NE->Epi
15) Purine synthesis requires
- Glycine, Aspartate
- Serine, Aspartate, Glycine
Correct answer: Glycine, Glutamine, Aspartate
16) Neonatal tyrosinemia
- Delay in p-hydroxyphenylpyruvate, serious disease, dietary treatment
- Delay in s-hydroxyphenylpyruvate, typically benign, dietary treatment
- Deficiency of homogenistate oxidase
Correct answer: Delay in p-hydroxyphenylpyruvate, typically benign, dietary treatment
17) GABA synthesis
- Glutamate carboxylase
- Glutamate
- Glutamate synthase
Correct answer: Glutamate decarboxylase
18) Untrue about PKU
- May be defective biopterin metabolism
- Toxic to cns
- 97% of children have low IQ
Correct answer: Uncommon
19) Tyrosinemia type 1
- Liver, kidney, peripheral ns problems, Tyrosine amino transferase deficiency
- Eye, skin, cns problems, tyrosine aminotransferase
Correct answer: Liver, kidney, peripheral ns problems, Fumarylacetoacetate hydrolase deficiency
20) Synthesis of porphyria requires
- Glutamine
- Aspartate
Correct answer: Glycine
21) How many ATP molecules does it take to produce one GSH
- 1
- 3
- 4
Correct answer: 2
22) Melatonin synthesis
- Trp->melatonin
- Serine->serotonin->Melatonin
Correct answer: Trp->serotonin->Melatonin
23) MSUD maple syrup urine disease
- Fumarylacetoacetate hydrolase deficiency
- Branched chain alpha ketoacid dehydrogenase upregulation
Correct answer: Branched chain alpha ketoacid dehydrogenase deficiency
24) Melanin synthesis
- Tryptophan -> dopa quinone-> melanin
- Dopa quinone->Tyrosine-> melanin
Correct answer: Tyrosine-> dopa quinone-> melanin
25) NAD metabolism
- Methionine, ATP, Methionine adenosyl transferase
- Tyrosine, PRPP, quinolate phosphoric bosyl transferase
Correct answer: Tryptophan, PRPP, quinolate phosphoric bosyl transferase
26) Catecholamines
- Epinephrine
- Norepinephrine
- Acetylcholine
Correct answer: Norepinephrine and Epinephrine
27) Histamine synthesis
- From tyrosine, requires aromatic acid decarboxylase
- From histidine, inhibited by aromatic acid decarboxylase
Correct answer: From histidine, requires aromatic acid decarboxylase
28) Carnotine synthesis requires
- Succinate, CO,` glycine
- Fe2+, alpha-kg, o2, SAM
- Fe2+, alpha-kg
Correct answer: Fe2+, alpha-kg, o2
29) Acetylcholine
- Tyrosine->Choline + acetyl CoA-> acetylcholine (requires SAM)
- Serine->Choline + acetyl CoA-> acetyl choline
- Serine->Choline-> acetyl choline (requires SAM)
Correct answer: Serine->Choline + acetyl CoA-> acetyl choline (requires SAM)
- Alpha KG defect
- Up regulation go phenylalanine hydroxylase
- Abundance of dihydropteridine reductase
Correct answer: Deficiency of phenylalanine hydroxylase
2) Alcaptonuria
- Clear urine
- Homogenistate oxidase hyperactivity
Correct answer: Homogenistate oxidase deficiency
3) Not part of a treatment for PKU
- Controlled low phenylalanine diet
- Oral tetrahydrobiopterin (THB)
- Long term
Correct answer: High phenylalanine diet
4) Creatine
- Formed ezymatically, entirely excreted in urine
- Formed nonezymatically, not excreted in urine
Correct answer: Formed nonezymatically, entirely excreted in urine
5) Serotonin synthesis
- Serine->serotonin
- Melatonin->serotonin
Correct answer: Trp->serotonin
6) Tyrosinemia type 2
- Liver, kidney, peripheral nerve defects. Deficiency of Fumarylacetoacetate hydrolase
Correct answer: Eye, skin defects. Deficiency of tyrosine amino acid transferase
7) Coenzyme A synthesis
- Phosphopantothenate + Serine-> Anp + CoA-SH
- Tryptophan + Cystiene -> Anp + CoA-SH
- Phosphopantothenate + SAM -> Anp + CoA-SH
Correct answer: Phosphopantothenate + Cystiene -> Anp + CoA-SH
8) Pyriminde biosynthesis requires
- Glycine, Glutamine, Aspartate
- Serine, Aspartate
Correct answer: Aspartate, Glutamine
9) Polyamines
- Putrescine , spermidine, ornithine. Derived from spermine and methionine
- Putrescine , spermidine. Derived from ornithine and methionine
- Putrescence, spermidine, spermine. Derived from ornithine and methionine
Correct answer: Putrescine, spermidine, spermine. Derived from ornithine and methionine
10) NO synthesis
- Arginine-> Citrulline-> NO
- Serine-> Citrulline + NO
Correct answer: Arginine-> Citrulline + NO
11) SAM synthesis
- Serine, Methionine and ATP
- Tryptophan, NAD
- Succinate, co2, glycine
Correct answer: Methionine and ATP
12) Classical albinism caused by
- Aromatic acid decarboxylase deficiency
- Tyrosinase overabundance
Correct answer: Tyrosinase deficiency
13) E-n-trimethyllysine conversion to
- SAM
- NAD
- Tryptophan
Correct answer: Carnitine
14) Catecholamine synthesis
- Phe->Dopa->Dopamine->NE->Epi
- Tyr->Dopamine->Dopa->NE->Epi
- Tyr->Dopa->Dopamine->Epi->NE
Correct answer: Tyr->Dopa->Dopamine->NE->Epi
15) Purine synthesis requires
- Glycine, Aspartate
- Serine, Aspartate, Glycine
Correct answer: Glycine, Glutamine, Aspartate
16) Neonatal tyrosinemia
- Delay in p-hydroxyphenylpyruvate, serious disease, dietary treatment
- Delay in s-hydroxyphenylpyruvate, typically benign, dietary treatment
- Deficiency of homogenistate oxidase
Correct answer: Delay in p-hydroxyphenylpyruvate, typically benign, dietary treatment
17) GABA synthesis
- Glutamate carboxylase
- Glutamate
- Glutamate synthase
Correct answer: Glutamate decarboxylase
18) Untrue about PKU
- May be defective biopterin metabolism
- Toxic to cns
- 97% of children have low IQ
Correct answer: Uncommon
19) Tyrosinemia type 1
- Liver, kidney, peripheral ns problems, Tyrosine amino transferase deficiency
- Eye, skin, cns problems, tyrosine aminotransferase
Correct answer: Liver, kidney, peripheral ns problems, Fumarylacetoacetate hydrolase deficiency
20) Synthesis of porphyria requires
- Glutamine
- Aspartate
Correct answer: Glycine
21) How many ATP molecules does it take to produce one GSH
- 1
- 3
- 4
Correct answer: 2
22) Melatonin synthesis
- Trp->melatonin
- Serine->serotonin->Melatonin
Correct answer: Trp->serotonin->Melatonin
23) MSUD maple syrup urine disease
- Fumarylacetoacetate hydrolase deficiency
- Branched chain alpha ketoacid dehydrogenase upregulation
Correct answer: Branched chain alpha ketoacid dehydrogenase deficiency
24) Melanin synthesis
- Tryptophan -> dopa quinone-> melanin
- Dopa quinone->Tyrosine-> melanin
Correct answer: Tyrosine-> dopa quinone-> melanin
25) NAD metabolism
- Methionine, ATP, Methionine adenosyl transferase
- Tyrosine, PRPP, quinolate phosphoric bosyl transferase
Correct answer: Tryptophan, PRPP, quinolate phosphoric bosyl transferase
26) Catecholamines
- Epinephrine
- Norepinephrine
- Acetylcholine
Correct answer: Norepinephrine and Epinephrine
27) Histamine synthesis
- From tyrosine, requires aromatic acid decarboxylase
- From histidine, inhibited by aromatic acid decarboxylase
Correct answer: From histidine, requires aromatic acid decarboxylase
28) Carnotine synthesis requires
- Succinate, CO,` glycine
- Fe2+, alpha-kg, o2, SAM
- Fe2+, alpha-kg
Correct answer: Fe2+, alpha-kg, o2
29) Acetylcholine
- Tyrosine->Choline + acetyl CoA-> acetylcholine (requires SAM)
- Serine->Choline + acetyl CoA-> acetyl choline
- Serine->Choline-> acetyl choline (requires SAM)
Correct answer: Serine->Choline + acetyl CoA-> acetyl choline (requires SAM)
