Lec 9: Intro To PROTEINS Flashcards
Building blocks of proteins
Amino acid
General Structure of Amino Acids
- An acid that is able to donate protons
- A base that is able to accept a proton
- Responsible for the function of the amino acid within the protein
- Carboxyl group
- Amino group
- R or sidechain
Smallest amino acid
Glycine
An imino acid
Proline
Amino acids which can’t be synthesized in the body
Essential
Examples of essential AA
- isoleucine
- methionine
- threonine
- histidine
- valine
- phenylalanine
- lysine
- leucine
Amino acids which can be synthesized in the boxy
Non-essential
Example of non-essential AA
Arginine
Amino acids which are required in growing children and pregnancy, but not essential in healthy adults
Semi-essential
• Inherited errors of metabolism
• an enzyme defect that inhibits the body’s ability to metabolize certain amino acids
Aminoacidopathies
The absence of the activity of phenylalanine hydroxylase enzyme.
Phenylketonuria
- Action of phenylalanine hydroxylase
- Effect of high levels of phenylalanine
- Describe urine of PKU patients
- PAH catalyzes conversion of phenylalanine to tyrosine
- High levels of Phenylalanine:
• retards mental development
• causes microcephaly
• leads to hypopigmentation
• become prone to seizures & eczema - Musty/Mousy odor
Low levels of enzyme fumarylacetoacetate hydrolase
Type 1 Tyrosinemia
What type of tyrosinemia?
• jaundice
• cabbage-like odor
• distended abdomen
• swelling of legs
Type 1 Tyrosinemia
Tyrosine aminotransferase deficiency
Type 2 Tyrosinemia