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
Q

Amyloid and Immunoglobulin are examples of which secondary structures?

A

Beta sheets

26
Q

Protein structure: overall 3D shape of protein

A

Tertiary Structure

27
Q

Pathology of Prion Diseases?

A

PrPc -alpha helices to pathogenic conformation PrPsc rich in Beta sheets

28
Q

Most common and most important degenerative disease of the brain: diffuse cerebral atrophy with dementia

A

Alzheimer Disease

29
Q

Characteristic feature of alzheimer disease?

A

senile plaques and neurofibrillary bundles (protein beta amyloid)

30
Q

what is the potential mediator of conformational transformation in alzheimer disease

A

apolipoprotein E

31
Q

amino acids found in collagen?

A

proline and glycine

32
Q

amino acids found in collagen?

A

proline and glycine

33
Q

what is X and Y in collagen structure?

A

X - Proline Y - hydroxyproline/hydroxylysine

34
Q

Type 1 collagen

A

Bone, skin, tendon, dentin, fascia, cornea, late wound repair

35
Q

Type II collagen

A

Cartilage (including hyaline), vitreous body, nucleus pulposus

36
Q

Type III collagen

A

(Reticulin) Skin, Blood Vessels, Uterus, Fetal tissue, Granulation tissue

37
Q

Type IV collagen

A

Basement membrane or basal lamina

38
Q

Type VII collagen

A

beneath stratified squamous epithelium

39
Q

Characteristics of Ehlers-Danlos Syndrome

A

hyperextensibility of skin, abnormal tissue fragility, increased joint mobility

40
Q

What type of collagen is defective in Osteogenesis imperfecta?

A

type I collagen

41
Q

Manifestations of osteogenesis imperfecta?

A

multiple fractures, blue sclerae, conductive hearing loss, dental imperfections

42
Q

What collagen is defective in Alport Syndrome?

A

Type IV collagen

43
Q

Manifestations of Alpory Syndrome?

A

Hematuria (main presenting sign), ocular lesions, sensorineural hearing loss, esrd

44
Q

What type of defect is seen in epidermolysis bullosa?

A

Type VII

45
Q

What is defective in Scurvy?

A

Hydroxylation of collagen requiring ascorbic acid

46
Q

Manifestations of scurvy?

A

Sore spongy gums, Loose teeth, poor wound healing. petechiae on skin and mucous membranes

47
Q

What collagen disease is characterized with kinky hair and growth retardation?

A

Menkes Disease

48
Q

In Menkes Disease, what dietary vitamin is deficient and wha enzyme is required for covalent crosslinking of collagen fibers?

A

Copper; lysyl oxidase

49
Q

Tissues where elastic recoil are needed?

A

lungs, large arteries, elastic ligaments, vocal cords, ligamentum flavum

50
Q

Components of elastin?

A

Proline and Lysine, little hydroxyproline, no hydroxylysine

51
Q

what precursor of elastin is deposited into an irregular fibrillin scsffold which id crosslinked by desmosine?

A

Tropoelastin

52
Q

What gene is mutated in Marfan Syndrome?

A

Fibrillin gene

53
Q

Manifestations of Marfan Syndrome?

A

Dolichostenomelia, Arachnodactyly, Aortic Dissection, Ectopia lentis

54
Q

What is the role of alpha 1 antitrypsin?

A

inhibits proteolytic enzyme from hydroloyzing and destroying proteins

55
Q

Compounds that increase the permeability of the inner mitochondrial membrane to protons

A

Uncouplers

56
Q

ATP inhibitor that directly inhibits ATP synthase (complex v)

A

ATP synthase inhibitors

57
Q

Inhibits oxidative phosphorylation by inhibiting the transporter of ADP into and ATP out of the mitochondrion

A

Atractyloside