Amino acid degradation

Glucogenic essential:

Alanine

Isoleucine

Phenylalanine

Histidine

Theronine

Tryptophan

Methionine

Valine

Acetyl CoA & Acetoacetyl CoA

Classified as: Ketogenic

Classified as: Glucogenic

Provide: Lipids, energy

Provide: Lipids, energy, glucose

Pyruvate, ocaloacetate, fumarate, a-ketogluterate & succinyl CoA:

Classified as: Ketogenic

Classified as: Glucogenic

Provide: Lipids, energy

Provide: Lipids, energy, glucose

Serine:

Is dehydrated to pyruvate

Can be converted to glycine

Is synthesized from 3-phosphoglycerate

Can be converted to glutamate

What is correct?

Alkaptonruia results from disturbances in tyrosine metabolism

�Mousy” odor of urine is characteristic in phenylketonuria

Maple syrup urine disease is a result of deficiency in branched chain amino acid aminotransferase

Tyrosinemia type 1:

Caused by a deficiency of fumaryl-acetoacetate hydrolase

It results in renal tubular acidosis

Treatment includes dietary restriction of asparagine

Choose true sentences:

Lysine is both keto- and glucogenic

A donor of a methyl group in resynthesis of methionine from Hcy is SAM

The excretion of FIGlu can be used in diagnosing a deficiency of folic acid

Asparagine synthetase:(usikker?)

AMP is one of the products of catalyzed reaction

Use glutamate as substrate

Convert oxaloacetate to asparagine

Tetrahydrobiopterin serves as a coenzyme in

Resynthesis of methionine to homocysteine

Hydroxylation of phenylalanine to tyrosine

Serotonin synthesis

Catecholamine synthesis

Glutamine:

Its conversion to glutamate is a source of the phosphate group

Its synthesis requires energy

Might be a source of alpha-ketoglutarate

Choose true sentences:

All branched-chain amino acids are both keto- and glucogenic

Dihydropteridine reductase deficiency may result in insufficient catecholamine synthesis

In homocystinuria, high plasma concentrations of methionine and cysteine are observed

Choose true sentences:

Histidine is oxidatively deaminated by histidase

Methionine can form fumarate

Albinism is an effect of defective metabolism in tyrosine

Homocystinuria

In this disease the level of cysteine in blood is elevated

The classic form is due to a deficiency in cystathionine synthase

The level of homocysteine in urine is high

Name and structure of amino acid with nonpolar side chain that forms α-ketoglutarate via glutamate.

Glycine

Glutamate

Glutamine

Glycine:

Can be converted to valine

Is a substrate of phenylalanine hydroxymethyl-transferase

Is glucogenic essential amino acid

None

Underline correctly:

Tyrosine synthesis needs presence of tetrahydrobiopterin

Maple syrup urine disease is due to a deficiency in branched-chain α-keto acid dehydrogenase

Catabolism of leucine yields acetyl CoA and propionyl CoA

Phenylketonuria:

Is caused by a deficiency of phenylalanine hydroxylase

Is characterized also by elevated levels of pyruvate, acetate and lactate

Is characterized also by hypopigmentation

Underline properly:

The conversion of serine to glycine requires the presence if tetrahydrofolate

Tyrosine is both ketogenic & glucogenic amino acid because its catabolism provides acetoacetate & fumarate

Urinary urocanic acid excretion is used as a diagnostic test to detect folic acid deficiency true

Underline correctly:

Methionine synthase catalyzes the reaction of methionine formation from:cysteine

Methionine synthase catalyzes the reaction of methionine formation from: homocysteine

The donor of the CH3 group in the synthesis of epinephrine is:S-adenosylhomocystein

The donor of the CH3 group in the synthesis of epinephrine is: S-adenosylmethionine

The synthesis of cysteine requires the presence of vitamine:tetrahydrofolate

The synthesis of cysteine requires the presence of vitamine:vitamin B6

Which of the sentences are true:

Glutamate can be synthesized by a reaction catalyzed by glutamate dehydrogenase,aminotransferase & glutamine synthetase

Serine may be a substrate for the synthesis of selenocysteine

Arginine is formed by the condensation of urea & ornithine

Underline properly:

Hyperphenylalaninemia may be due to deficiency of : tyrosine hydroxylase

Hyperphenylalaninemia may be due to deficiency of :dihydropteridine reductase

Alkaptonuria results from a disturbance of: tyrosine catabolism

Maple syrup disease is due to: dihydrofolate reductase deficiency

Maple syrup disease is due to: branched-chain amino acid catabolism

Amino acid degradation

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