AA Flashcards

1
Q

Enzymes defects that inhibits the body’s ability to metabolize certain amino acids

A
PKU
Tyrosinemia	
Alkaptonuria
MSUD
Isovaleric Acidemia
Homocystinuria
Citrullinemia
Arginosuccinic Aciduria
Cystinuria
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2
Q

Phenylketonuria
Absence of _
_ of urine

A
phenylalanine hydroxylase (PAH)
Musty odor
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3
Q

Laboratory Tests Phen

A

Guthrie test

Microfluorometric assay

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4
Q

Semi quantitative bacterial inhibition assay

Uses phenylalanine to facilitate bacterial growth (____ and___).

A

Guthrie test

B. subtilis and β-2-thienylalanine

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5
Q

Type II Tyrosinemia

A

↓ Tyrosine aminotransferase

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6
Q

Type III Tyrosinemia

A

↓ 4-hydroxyphenylpyruvate dioxygenase

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7
Q

Alkaptonuria
Lack of–

A

Lack of homogentisate oxidase

↑ homogentesic acid in urine

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8
Q

Type I Tyrosinemia

A

↓ fumarylacetoacetate hydrolase

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9
Q
Maple Syrup Urine Disease
\_\_\_
\_\_
_
-
\_\_\_odor of urine
A

↓ branched-chain α-ketoacid decarboxylase
↑ Isoleucine
↑ Leucine
↑ Valine

Burnt sugar

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10
Q

Map Syr test

A

Modified Guthrie test

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11
Q

Modified Guthrie test

uses branched chain ___ to facilitate bacterial growth (containing bbbbb and cvcvc).

A

α-ketoacid
B. subtilis
4-azaleucine

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12
Q

Isovaleric Acidemia
Deficiency of vvvvvvv
vvvvvv
vvvvv odor

A

Deficiency of isovaleryl-CoA dehydrogenase
↑ Isovaleric acid
Sweaty feet odor

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13
Q

Homocystinuria

Lack of vvvv
vvvv
vvvv

A

cystathionine β- synthetase
↑ Homocysteine
Methionine

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14
Q

Homocystinuria test

Uses vvv to facilitate bacterial growth (containing B. subtilis and vvvv).

A

Modified Guthrie test

Uses methionine to facilitate bacterial growth (containing B. subtilis and L-methionine sulfoximime).

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15
Q

Type I citrullinemia

Lack of vvvvv (ASAS)

A

Type I citrullinemia

Lack of arginonosuccinic acid synthetase (ASAS)

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16
Q

Mutation of the gene that encodes for protein citrin

A

Type II citrullinemia

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17
Q

Argininosuccinic aciduria

Lack of

A

argininosuccinic acid lyase (ASL)

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18
Q

Defect in amino acid transport system

Inadequate reabsorption of cystine in the kidneys

A

Cystinuria

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19
Q

Disintegration of protein to amino acids
degrades extracellular proteins
intra

A

Lysosomal pathway

Cytosolic pathway

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20
Q

Levels of protein structure

4

A

Primary
Secondary
Tertiary
Quarternary

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21
Q

Amino acids in a specific sequence

Regularly repeating structures stabilized by hydrogen bonds between the amino acids within the protein

Overall conformation (fold) of the protein molecule
Due to interaction of side chains (e.g. ionic) 

Interaction of more than 1 protein molecule or subunits

A

Primary
Secondary
Tertiary
Quarternary

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22
Q

Classification by Protein Functions 10

A
Enzymes 
Hormones 
Transport proteins
Immunoglobulins (antibodies)
Structural proteins
Storage proteins
Energy Source
Osmotic force
Homeostasis
Acid-Base Balance
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23
Q

Contain peptide chains composed of only amino acids.

May be globular(_ _ ) or fibrous ()

A

Simple Proteins

May be globular (hormone, enzymes, transport) or fibrous (structural)

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24
Q

Conjugated Proteins

4

A

Metalloprotein
Lipoprotein
Mucoprotein and glycoprotein
Nucleoprotein

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25
Q

With higher carbohydrate -

10%-40% carbohydrate -

Nucleic acids attached-

A

Mucoproteins or proteoglycans - Mucin

Glycoproteins - Haptoglobin and α1-antitrypsin

Nucleoproteins - Chromatin

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26
Q

Plasma Proteins 2

A

Albumin

Globulin
α1-Globulins
α2- Globulins
β-Globulins
γ-Globulins
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27
Q

Indicator of nutrition
Binds thyroid hormones (_ _)
Binds retinol-binding protein

A

Prealbumin

T3, T4

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28
Q

Binds bilirubin, steroids, fatty acids

Major contributor to _ _

A

Albumin

oncotic pressure

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29
Q

α1-Globulins

A
α1-
Antichymotrypsin 
Antitrypsin
 Lipoprotein
 Fetoprotein
 Acid glycoprotein
Inter-α-trypsin inhibitor
Gc-globulin
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30
Q

α1 - Antitrypsin

and

A

Acute phase reactant (α1 - Acid glycoprotein
)
Protease inhibitor

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31
Q

α1 - Fetoprotein

A

Principal fetal protein

↑ spina bifida, ↓ - Down syndrome

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32
Q

Transport lipids (HDL)

A

α1 – Lipoprotein

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33
Q

Inhibits serine proteinases

-
Gc-globulin

A

α1 - Antichymotrypsin
Inter-α-trypsin inh.
-
Transports Vit. D and binds actin

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34
Q

α2-Globulins

3

A

Haptoglobins - Acute Phase reactant, Binds Hgb.

Ceruloplasmin
α2 - Macroglobulin

35
Q

Ceruloplasmin
Contains __
↓ - _ _

A

Contains copper

↓ - Wilson’s disease. Menkes synd

36
Q

Inhibits protease

A

α2 - Macroglobulin

37
Q

β-Globulins

8

A
Pre-β-lipoprotein
β-Lipoprotein
Trasferrin
Hemopexin
β2-Microglobulin
C4, C3, C1q complement
Fibrinogen
C-reactive protein
38
Q
Transports lipids (VLDL triglyceride)
Transports lipids (LDL cholesterol)
A

Pre-β-lipoprotein

β-Lipoprotein

39
Q

Transport Iron,↑-IDA,↓ Hemochromatosis

A

Trasferrin

40
Q

Acute phase reactant, Binds heme

A

Hemopexin

41
Q

Component of HLA molecules

A

β2-Microglobulin

42
Q

Immune response (Opsonins)

A

C4, C3, C1q complement

43
Q

Precursor of fibrin clot

A

Fibrinogen

44
Q

Acute phase reactants

Promotes phagocytosis

A

C-reactive protein

45
Q
γ-Gamma-Globulins
Immunoglobulin G
Immunoglobulin A
M
EE
A
Antibodies
Antibodies in secretions
Antibodies in early response
Antibodies (reagen, allergy)
Surface antibody
46
Q

Other Proteins 7

A

Myoglobin

Troponin (cTn)

Fibronectin
Fetal fibronectin (fFN)

Cross-Linked
C-Telopeptides

β-Trace Protein

Cystatin C

Amyloid

47
Q

Cellular interaction
Placental adherence to the uterus
↑ - Preterm labor and delivery

A
Fibronectin
Fetal fibronectin (fFN)
48
Q

Cardiac marker for acute coronary syndrome

A

Troponin (cTn)

49
Q

Oxygen carrier in muscles
Cardiac marker (AMI)
↑ 2-3 hrs of onset, peak at 8-12 hrs

A

Myoglobin

50
Q

Proteolytic fragment of collagen I

Marker of bone resorption

A

Cross-Linked

C-Telopeptides

51
Q

Syn: Prostaglandin D synthase

Marker for CSF leakage

A

β-Trace Protein

52
Q

Cysteine proteinase inhibitor

Marker for kidney function (GFR)

A

Cystatin C

53
Q

Fibrous protein aggregates formed from alteration of β pleated sheats

↑ Amyloidoses

A

Amyloid

54
Q

Total Protein Abnormalities

A

TAG

55
Q

N down up

A

Hepatic Damage
Cirrhosis β-γ bridging
Hepatitis ↑ γ-globulins

56
Q

down down up

Acute -
Chronic -

A

Infections
Acute - α1 , α2 globulins
Chronic - ↑ α1, α2 γ, globulins

57
Q

down down N

A

Inadequate diet
Nephrotic syndrome
↑α2,β-globulins;↓γ-globulins

58
Q

Down N Down

A

Immunodeficiency syndrome

59
Q

All UP

A

Dehydration

60
Q

Up and Up

A

Multiple myeloma
Monoclonal &
Polyclonal gammopathies

61
Q

Method of Analysis Total Protein

A

Kjeldahl
Refractometry
Biuret
Dye Binding

62
Q

Method of Analysis Albumin and Globulin

A

Salt Precipitation
Dye Binding (Methyl orange, HABA, BCG, BCP)
Electrophoresis (Coomassie blue)

63
Q

TP Reference method. Assume average ____ of 16%

A

Kjeldahl

nitrogen content

64
Q

Measurement of refractive index due to solutes in serum

A

Refractometry

65
Q

Formation of violet-colored chelate between VVV ions and VVVV

A

Biuret
Cu2+
peptide bonds

66
Q

Protein binds to dye and causes a spectral shift in the absorbance maximum

A

Dye Binding

67
Q

Kjeldahl 3

A

Kjeldahlization
Ammonia measurement-Nessler’s reaction (HgI2/KI)
-Berthelot reaction

68
Q

conversion of nitrogen to ammonia

Nitrogen - - NH3

A

Kjeldahlization – H2SO4

Nitrogen H2SO4 NH3

69
Q

Nessler’s reaction

Ammonia + Nessler’s rgt ___ — (NH2Hg2I)

A

Ammonia + Nessler’s rgt Gum ghatti  Yellow solution (NH2Hg2I)

70
Q

Berthelot reaction

Ammonia + alkaline hypochlorite ___ –

A

Na nitroprusside

Indophenol blue

71
Q
Biuret
Composition
 – breaks the peptide bonds
– keeps copper in solution
– stabilizes cupric ions
A

Cupric ions
Tartrate salt
Potassium iodide

72
Q

Dye binding 5

A
Bromphenol blue
Ponceau S
Amido black 10B
Lissamine green
Coomassie brilliant blue
73
Q

Globulins are precipitated

Albumin in supernatant is quantitated by biuret reaction

A

Salt Precipitation

74
Q

Dye Binding

4

A

Methyl orange
HABA
BCG (Bromcresol green)
BCP (Bromcresol purple)

75
Q

Nonspecific for Albumin

A

Methyl orange

76
Q

HABA

Many Interferences

A

Many Interferences (salicylates, bilirubin)

77
Q

Sensitive, Most commonly used dye

A

Bromcresol green

78
Q

Specific, Sensitive and Precise

A

Bromcresol purple

79
Q

Proteins separated based on electric charge densities

A

Electrophoresis

80
Q

Electrophoresis (support media)

A

Cellulose acetate/agarose gel

81
Q

Electrophoresis

After separation, protein fractions are immersed in acid solution then stained by dyes

A

(Coomassie blue)

scenned in densitometer

82
Q

Uses higher voltage couple with a cooling system and more concentrated buffer

A

Electrophoresis (High Resolution)

83
Q

It is the separation of molecules which takes place in silica capillaries.

A

Capillary Electrophoresis

84
Q

Separates proteins on the basis of pI.

Uses constant power and __ or _ which contains a pH gradient.

pH gradient: ___

A

polyacrylamide or agarose gel
3.5 to 10