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
What type of tyrosinemia?
• mental retardation
• excessive tearing
• photophobia
• eye pain & redness
• painful skin lesions (palms & soles)
Type 2 Tyrosinemia
Deficiency of 4-hydroxyphenylpyruvate dioxygenase
Type 3 Tyrosinemia
What type of tyrosinemia?
• mental retardation
• periodic loss of balance and coordination
Type 3 Tyrosinemia
What type of tyrosinemia?
- Mild form
- Most severe form
- Type III
- Type I
For detection of Tyrosinemia
Tandem Mass Spectrometry
Deficiency of homogentisate oxidase
Alkaptonuria
• urine turns black when mixed with air
• dark spots on sclera
• ochronosis
Alkaptonuria
- high levels of thyroxine and phenylalanine —> secreted in urine
- ochronosis: darkening of cartilage tissues due to deposition of pigment
Action of Homogentisate oxidase
For the metabolism of thyroxine & phenylalanine
Absence of branched-chain alpha keto acid decarboxylase
Maple Syrup Urine Disease
Maple Syrup Urine Disease
- Describe urine
- Infants born with this develop ___ (symptoms).
- Treatment
- Test for detection
- Burnt sugar odor (including skin & breath)
- Lethargy, vomiting, lack of appetite, failure to thrive
- Avoidance of branched-chain keto acid in diet
- Modified Guthrie Test
• inhibitor: 4-azaleucine
Linear polymers of amino acids
Protein
Functions of Protein
- regulate metabolism
- facilitate muscle contraction
- provide structural framework
- as transport proteins
- component of the immune system
- acid-base balance
- water distribution maintenance
- DNA produces RNA template
- formation of mRNA strand
- mRNA is sent to cytoplasm
- process takes place in nucleus
Transcription
- formation of RNA into protein
- takes place in cytoplasm
Translation
Protein Structures
- Folding of short contiguous segments of polypeptide into geometrically ordered units
- A sequence of a chain of amino acids
- Results from the interaction of more than one protein molecule/sub-units.
- Three-dimensional shape of protein. Either globular or fibrous.
- Secondary structure
- Primary structure
- Quarternary structure
- Tertiary structure
Secondary structure
- Most common secondary structure. Spiral structure.
- A zigzag or pleated pattern.
- Alpha helix
- Beta-pleated sheet
Four major types of plasma proteins
- albumin
- globulin
- fibrinogen
- others..
- it migrates before albumin in electrophoresis
- transport protein for t3 and t4
Pre-Albumin
- Synthesized in the liver
- responsible for most of the colloid osmotic pressure
- present in highest concentration in the plasma
Albumin
A protein for:
- cell adhesion
- tissue differentiation
- growth
- wound healing
Fibronectin
- Insoluble fibrous protein aggregates that are formed due to the alteration in their secondary structure (beta pleated sheets)
- an abnormal protein found in different diseases
Amyloid