Amino Acids Metabolism: Tyrosine, Phenylketonuria, Albinism

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25 Questions

What is the metabolic role of tyrosine in the body?

Synthesis of dopamine

Which amino acid is synthesized from phenylalanine in the liver?

Tyrosine

Which amino acid is an essential amino acid?

Phenylalanine

What is the main function of melanin synthesized from tyrosine?

Protecting the skin from UV rays

What is the metabolic disorder characterized by an absence of the enzyme required for phenylalanine metabolism?

Phenylketonuria

Which of the following is a feature of albinism?

Deficiency in melanin production

What is the main cause of Hyperphenylalaninemia type 1?

Genetic deficiency of phenylalanine hydroxylase

What is the alternative pathway for oxidation of phenylalanine in Hyperphenylalaninemia type 1?

Conversion into phenylacetate, phenylpyruvate, and phenylactate

In Hyperphenylalaninemia type 1, what is the clinical manifestation related to skin color?

Light color of hair & skin

What is the diagnosis test for phenylketonuria?

Urine ferric chloride test

What is the recommended treatment for phenylketonuria in children?

Low concentrations of phenylalanine in the diet after age 6 years

What is the main cause of albinism?

Genetic deficiency of tyrosinase

What causes mental retardation and seizures in Hyperphenylalaninemia type 1?

Competitive inhibition of pyruvate transport to mitochondria by phenylpyruvate

What is the specific clinical manifestation in alkaptonuria related to urine color?

Blackish-brown urine when fresh

What is the most likely cause of ochronosis and a form of arthritis in alkaptonuria?

Build-up of homogentisate in connective tissues

What is the main biological function of protein synthesis in adults?

To replace constantly turning over proteins

Which compounds are synthesized from amino acids?

Purines and pyrimidines

What is the role of Krebs cycle in amino acid metabolism?

It provides missing elements for protein synthesis

Which enzyme is responsible for cleaving the basic amino acids from the C-terminal end of the peptide during protein digestion?

Pancreatic carboxypeptidases

What is the main function of large peptides and free amino acids in protein digestion?

Replacement of constantly turning over proteins

What is the main fate of the absorbed amino acids?

Incorporation into tissue proteins

Where does oxidative deamination of amino acids mainly occur?

Liver and kidney

What is the significance of L-glutamate dehydrogenase in oxidative deamination?

Conversion of alpha-ketoglutaric acid to glutamic acid

What is the primary metabolic fate of non-essential amino acids in the body?

Incorporation into tissue proteins

What is the main function of transamination in amino acid metabolism?

Transfer of the amino group to a suitable keto acid acceptor

This quiz covers the functions of tyrosine amino acid, phenylketonuria including its etiology, clinical presentation, diagnosis, and treatment, as well as the definition and etiology of albinism. It also explores the metabolic characteristics of phenylalanine and tyrosine amino acids.

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