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Chemistry Module 1 > Amino Acids > Flashcards

Amino Acids Flashcards

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0
Q

Means that amino acids contain both acidic and basic groups

A

Amphoteric (zwitterion)

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1
Q

Amino acids may have a positive, negative, or neutral charges, depending on the _____

A

pH

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2
Q

pH>7.4 , when a base is added to the amino acid resulting molecule has a _______ charge

A

Negative

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3
Q

pH=7.4 , internal transfer of a hydrogen ion from the -COOH group to the -NH2 group results in a _________ charge

A

Neutral

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4
Q

pH<7.4 decrease in pH by adding an acid results in a _______ charge

A

Positive

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5
Q

Defined as the pH at which net charge is zero; i.e. There are equal numbers of positive and negative charges

A

Isoelectric point

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6
Q

If pH > pl

A

Amino acid has a negative charge

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7
Q

If pH < pl

A

Amino acid has a positive charge

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8
Q

Proteins act in vivo as a _______ _______ due to their acid-base properties

A

Mild buffer

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9
Q

Four essential amino acids

A

Leucine. Isoleucine, Valine, Methionine, Tryptophan, Phenylalanine, Threonine, Lysine

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10
Q

Formed when the alpha amino group of one amino acid is covalently linked with the alpha-carboxyl group of a second amino acid

A

A peptide bond

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11
Q

The formation of glucose from non-carbohydrate sources, such as amino acids, glycerol, or fatty acids

A

Gluconeogenesis

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12
Q

Starting and ending products of gluconeogenesis

A

S: amino acids/glycerol/fatty acids
E: glucose

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13
Q

The process of amino group removal or transfer to make different amino acids

A

Transamination

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14
Q

Starting and ending products of transamination (2 of them)

A

S: glutamic acid/Oxaloacetate
E: alanine/aspartate

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15
Q

The removal of the amino group, forming toxic ammonia

A

Deamination

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16
Q

Starting and ending products of deamination

A

S: Ammonia
E: Urea

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17
Q

Organ in which the metabolic/catabolic pathways occur in the body

A

liver

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18
Q

occurs when the plasma levels of the amino acid are normal, but the renal reabsorptive mechanism is defective

A

Renal Aminoaciduria

19
Q

______ plasma levels and _______ urine levels of amino acids in renal aminoaciduria

A

normal; increased

20
Q

Defect in renal tubular reabsorption of cystine, ornithine, lysine, and arginine; most common error of amino acid transport

A

cystinuria

21
Q

Three primary renal aminoacidurias

A

cystinuria, hartnup disease, and fanconi syndrome

22
Q

prone to renal calculi made of cystine; cystine crystals on urine microscopic

A

lab findings in cystinuria

23
Q

Increased urinary excretion of neutral monocarboxylic amino acids

A

Hartnup Disease

24
Q

Patients have a nicotinamide (vit B) deficiency, so they can have pellagra (red, scaly rash), plus neurological and muscular abnormalities

A

Clinical features of hartnup disease

25
Q

renal dysfunction of the proximal convoluted tubule

A

Fanconi Syndrome

26
Q

polyuria, dehydration, hypokalemia, acidosis, osteomalacia, cannot reabsorb necessary nutrients, bowing of the legs, low weight/height, abdominal distention, enlargement in wrist and ankle joints

A

clinical features of fanconi syndrome

27
Q

plasma level of one or more amino acids exceeds the rena threshold

A

overflow aminoaciduria

28
Q

______ plasma levels and _______ urine levels of amino acids in overflow aminoaciduria

A

Increased; Increased

29
Q

Due to a deficiency or absence of phenylalanine hydroxylase enzyme

A

Phenylketonuria (PKU)

30
Q

spastic movements and seizures, sever mental retardation if untreated, eczema, feeding difficulties, vomiting, delayed mental/social development, hypopigmentation, Increased serum phenylalanine, Decreased serum tyrosine, increased urine phenylpyruvic acid

A

Clinical features of PKU

31
Q

Due to fumarylacetoacetase deficiency

A

Type I Tyrosinemia

32
Q

causes cirrhosis and renal damage leading to fanconi, elevated serum and urine tyrosine levels, elevated methionine in serum

A

Clinical features of Type I tyrosinemia

33
Q

Due to tyrosine aminotransferase deficiency

A

Type II Tyrosinemia

34
Q

skin lesions, ocular abnormalities (corneal dystrophy), elevated serum and urine levels of tyrosine, tyrosine crystals in urine, serium methionine NOT increased

A

clinical features of Type II Tyrosinemia

35
Q

due to 4-hydroxyphenylpyruvate dioxygenase deficiency

A

Type III Tyrosinemia

36
Q

mild mental retardation, seizures, periodic loss of balance and coordination, increased serum tyrosine

A

Type III Tyrosinemia

37
Q

caused by a defect in the enzyme homogentisic acid oxidase

A

Alkaptonuria

38
Q

homogentisic acid inreases in serum and urine, urine turns brown/black when exposed to light and air or when alkali is added, ocular ochronosis

A

clinical features of Alkaptonuria

39
Q

most commonly caused by a defect of CBS (cystathionine beta synthase)

A

homocystinuria

40
Q

both methionine and homocystine are increased in serum and urine, ocular (lens displacement) and skeletal abnormalities (knock knee), cardiovascular problems (thromboli due to sticky platelets), pigeon chest

A

clinical features of homocystinuria

41
Q

due to a defect in the enzyme alpha-ketoacid decarboxylase; also known as branched chain aminoaciduria due to excretion of the branched chain amino acids valine, leucine, and isoleucine

A

Maple Syrup Urine Disease (MSUD)

42
Q

symptoms include hypoglycemia, ketoacidosis (blood and urine), lethargy, poor appetite, vomiting, convulsions, detected by the 11th day of life, maple syrup odor, increased ketone bodies

A

MSUD clinical features

43
Q

Amino acids can be measured using 3 screening methods

A

Thin layer chromatography (TLC), photometric screening tests, and Guthrie test

44
Q

three quantitative testsf for the presence of aminoacidurias

A

ion exchange chromatography, gas liquid chromatography (GLC), and high performance liquid chromatography (HPLC)

Chemistry Module 1 (18 decks)

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