Biochemistry Flashcards

Proteins

1
Q

Each amino acids has the ff structures

A

carboxyl group, amino group, distinctive side chain

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2
Q

A nonpolar amino acid with the smallest side chain and is used in first step of heme synthesis

A

Glycine

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3
Q

A nonpolar amino acid which is a carrier of ammonia and of the carbons of pyruvate from skeletal muscle to liver

A

Alanine

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4
Q

What are the branched-chain amino acids whose metabolites accumulate in maple syrup urine disease

A

Valine, Leucine, Isoleucine

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5
Q

Precursor of tyrosine?

A

Phenylalanine

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6
Q

Precursor for Niacin, Serotonin, Melatonin

A

Tryptophan

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7
Q

Precursor of homocysteine?

A

Methionine

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8
Q

An imino acid that contributes to the fibrous structure of collagen and interrupts alpha helices in globular proteins

A

Proline

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9
Q

A nonpolar amino acid that is a major inhibitory neurotransmitter in the spinal cord

A

Glycine

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10
Q

A nonpolar amino acid which, in together with glycine, constitutes a major fraction of free acids in the blood

A

Alanine

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11
Q

Tyrosine is the precursor of these compounds:

A

Phenylalanine, Tyrosine, L dopa, Dopamine, Norepinephrine, Epinephrine, Thyroxine, Melanin

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12
Q

What amino acid is the site for N linked glycosylation of proteins?

A

Asparagine

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13
Q

Is deaminated by glutaminase resulting in the formation of ammonia, and is a major carrier of nitrogen to the liver from peripheral tissues

A

Glutamine

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14
Q

An amino acid which contains a sulfydryl group that is an active part of many enzymes

A

Cysteine

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15
Q

An amino acid which participates in the biosynthesis of coenzyme A?

A

Cysteine

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16
Q

Two amino acids that are negatively charged at neutral pH because of carboxylate group

A

Aspartate and Glutamate

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17
Q

Precursors for GABA and glutathione

A

Glutamate

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18
Q

Two amino acids that are positively charged at physiologic pH

A

Arginine and Lysine

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19
Q

Precursor of histamine

A

Histidine

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20
Q

Precursor of creatinine, urea, and nitric oxide

A

Arginine

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21
Q

Proteim Structure: determined by amino acid sequence

A

Primary Structure

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22
Q

Peptide bonds attaches which 2 structures?

A

alpha amino group to alpha carbonyl

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23
Q

Protein structure: folding of short (3-30) contiguous segments which are stabilized by hydrogen bonding

A

secondary structures

24
Q

Keratin and Hemoglobin are examples of which secondary structures?

A

Alpha Helix

25
Amyloid and Immunoglobulin are examples of which secondary structures?
Beta sheets
26
Protein structure: overall 3D shape of protein
Tertiary Structure
27
Pathology of Prion Diseases?
PrPc -alpha helices to pathogenic conformation PrPsc rich in Beta sheets
28
Most common and most important degenerative disease of the brain: diffuse cerebral atrophy with dementia
Alzheimer Disease
29
Characteristic feature of alzheimer disease?
senile plaques and neurofibrillary bundles (protein beta amyloid)
30
what is the potential mediator of conformational transformation in alzheimer disease
apolipoprotein E
31
amino acids found in collagen?
proline and glycine
32
amino acids found in collagen?
proline and glycine
33
what is X and Y in collagen structure?
X - Proline Y - hydroxyproline/hydroxylysine
34
Type 1 collagen
Bone, skin, tendon, dentin, fascia, cornea, late wound repair
35
Type II collagen
Cartilage (including hyaline), vitreous body, nucleus pulposus
36
Type III collagen
(Reticulin) Skin, Blood Vessels, Uterus, Fetal tissue, Granulation tissue
37
Type IV collagen
Basement membrane or basal lamina
38
Type VII collagen
beneath stratified squamous epithelium
39
Characteristics of Ehlers-Danlos Syndrome
hyperextensibility of skin, abnormal tissue fragility, increased joint mobility
40
What type of collagen is defective in Osteogenesis imperfecta?
type I collagen
41
Manifestations of osteogenesis imperfecta?
multiple fractures, blue sclerae, conductive hearing loss, dental imperfections
42
What collagen is defective in Alport Syndrome?
Type IV collagen
43
Manifestations of Alpory Syndrome?
Hematuria (main presenting sign), ocular lesions, sensorineural hearing loss, esrd
44
What type of defect is seen in epidermolysis bullosa?
Type VII
45
What is defective in Scurvy?
Hydroxylation of collagen requiring ascorbic acid
46
Manifestations of scurvy?
Sore spongy gums, Loose teeth, poor wound healing. petechiae on skin and mucous membranes
47
What collagen disease is characterized with kinky hair and growth retardation?
Menkes Disease
48
In Menkes Disease, what dietary vitamin is deficient and wha enzyme is required for covalent crosslinking of collagen fibers?
Copper; lysyl oxidase
49
Tissues where elastic recoil are needed?
lungs, large arteries, elastic ligaments, vocal cords, ligamentum flavum
50
Components of elastin?
Proline and Lysine, little hydroxyproline, no hydroxylysine
51
what precursor of elastin is deposited into an irregular fibrillin scsffold which id crosslinked by desmosine?
Tropoelastin
52
What gene is mutated in Marfan Syndrome?
Fibrillin gene
53
Manifestations of Marfan Syndrome?
Dolichostenomelia, Arachnodactyly, Aortic Dissection, Ectopia lentis
54
What is the role of alpha 1 antitrypsin?
inhibits proteolytic enzyme from hydroloyzing and destroying proteins
55
Compounds that increase the permeability of the inner mitochondrial membrane to protons
Uncouplers
56
ATP inhibitor that directly inhibits ATP synthase (complex v)
ATP synthase inhibitors
57
Inhibits oxidative phosphorylation by inhibiting the transporter of ADP into and ATP out of the mitochondrion
Atractyloside