Lec 9: Intro To PROTEINS Flashcards

1
Q

Building blocks of proteins

A

Amino acid

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2
Q

General Structure of Amino Acids

  1. An acid that is able to donate protons
  2. A base that is able to accept a proton
  3. Responsible for the function of the amino acid within the protein
A
  1. Carboxyl group
  2. Amino group
  3. R or sidechain
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3
Q

Smallest amino acid

A

Glycine

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4
Q

An imino acid

A

Proline

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5
Q

Amino acids which can’t be synthesized in the body

A

Essential

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6
Q

Examples of essential AA

A
  • isoleucine
  • methionine
  • threonine
  • histidine
  • valine
  • phenylalanine
  • lysine
  • leucine
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7
Q

Amino acids which can be synthesized in the boxy

A

Non-essential

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8
Q

Example of non-essential AA

A

Arginine

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9
Q

Amino acids which are required in growing children and pregnancy, but not essential in healthy adults

A

Semi-essential

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10
Q

• Inherited errors of metabolism
• an enzyme defect that inhibits the body’s ability to metabolize certain amino acids

A

Aminoacidopathies

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11
Q

The absence of the activity of phenylalanine hydroxylase enzyme.

A

Phenylketonuria

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12
Q
  1. Action of phenylalanine hydroxylase
  2. Effect of high levels of phenylalanine
  3. Describe urine of PKU patients
A
  1. PAH catalyzes conversion of phenylalanine to tyrosine
  2. High levels of Phenylalanine:
    • retards mental development
    • causes microcephaly
    • leads to hypopigmentation
    • become prone to seizures & eczema
  3. Musty/Mousy odor
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13
Q

Low levels of enzyme fumarylacetoacetate hydrolase

A

Type 1 Tyrosinemia

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14
Q

What type of tyrosinemia?

• jaundice
• cabbage-like odor
• distended abdomen
• swelling of legs

A

Type 1 Tyrosinemia

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15
Q

Tyrosine aminotransferase deficiency

A

Type 2 Tyrosinemia

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16
Q

What type of tyrosinemia?

• mental retardation
• excessive tearing
• photophobia
• eye pain & redness
• painful skin lesions (palms & soles)

A

Type 2 Tyrosinemia

17
Q

Deficiency of 4-hydroxyphenylpyruvate dioxygenase

A

Type 3 Tyrosinemia

18
Q

What type of tyrosinemia?

• mental retardation
• periodic loss of balance and coordination

A

Type 3 Tyrosinemia

19
Q

What type of tyrosinemia?

  1. Mild form
  2. Most severe form
A
  1. Type III
  2. Type I
20
Q

For detection of Tyrosinemia

A

Tandem Mass Spectrometry

21
Q

Deficiency of homogentisate oxidase

A

Alkaptonuria

22
Q

• urine turns black when mixed with air
• dark spots on sclera
• ochronosis

A

Alkaptonuria

  • high levels of thyroxine and phenylalanine —> secreted in urine
  • ochronosis: darkening of cartilage tissues due to deposition of pigment
23
Q

Action of Homogentisate oxidase

A

For the metabolism of thyroxine & phenylalanine

24
Q

Absence of branched-chain alpha keto acid decarboxylase

A

Maple Syrup Urine Disease

25
Q

Maple Syrup Urine Disease

  1. Describe urine
  2. Infants born with this develop ___ (symptoms).
  3. Treatment
  4. Test for detection
A
  1. Burnt sugar odor (including skin & breath)
  2. Lethargy, vomiting, lack of appetite, failure to thrive
  3. Avoidance of branched-chain keto acid in diet
  4. Modified Guthrie Test
    • inhibitor: 4-azaleucine
26
Q

Linear polymers of amino acids

A

Protein

27
Q

Functions of Protein

A
  • regulate metabolism
  • facilitate muscle contraction
  • provide structural framework
  • as transport proteins
  • component of the immune system
  • acid-base balance
  • water distribution maintenance
28
Q
  • DNA produces RNA template
  • formation of mRNA strand
  • mRNA is sent to cytoplasm
  • process takes place in nucleus
A

Transcription

29
Q
  • formation of RNA into protein
  • takes place in cytoplasm
A

Translation

30
Q

Protein Structures

  1. Folding of short contiguous segments of polypeptide into geometrically ordered units
  2. A sequence of a chain of amino acids
  3. Results from the interaction of more than one protein molecule/sub-units.
  4. Three-dimensional shape of protein. Either globular or fibrous.
A
  1. Secondary structure
  2. Primary structure
  3. Quarternary structure
  4. Tertiary structure
31
Q

Secondary structure

  1. Most common secondary structure. Spiral structure.
  2. A zigzag or pleated pattern.
A
  1. Alpha helix
  2. Beta-pleated sheet
32
Q

Four major types of plasma proteins

A
  • albumin
  • globulin
  • fibrinogen
  • others..
33
Q
  • it migrates before albumin in electrophoresis
  • transport protein for t3 and t4
A

Pre-Albumin

34
Q
  • Synthesized in the liver
  • responsible for most of the colloid osmotic pressure
  • present in highest concentration in the plasma
A

Albumin

35
Q

A protein for:

  • cell adhesion
  • tissue differentiation
  • growth
  • wound healing
A

Fibronectin

36
Q
  • 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
A

Amyloid