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Biochem 2nd sem > Protein Metab 2 > Flashcards

Protein Metab 2 Flashcards

(73 cards)

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0
Q

Phenylalanine and tyrosine metabolic pathway

Major electron donor

A

NADPH

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1
Q

Phenylalanine and tyrosine metabolic pathway

Minor electron donor
Coenzyme
Carries reducing equivalents from NADH

A

Tetrahydrobioptrein THD

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2
Q

Phenylalanine and tyrosine metabolic pathway

Converts DHB to THB

A

DHB reductase

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3
Q

Phenylalanine and tyrosine metabolic pathway

Derivatives

A

Thyroid hormone
Melanin
Morepinephrine

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4
Q

Phenylketonuria

Three metabolites

A

Phenylpyruvic acid
Phenyllactate
Phenylacetate

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5
Q

Phenylketonuria

Urine odor

A

Mousy

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6
Q

Phenylketonuria

Mental retardation

A

Accumulation of phenylalanine in brain

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7
Q

Phenylketonuria

Inhibition of tyrosine hydroxylation to form melanin

A

Hypopigmentation

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8
Q

Phenylketonuria

No cathecolamines/ serotonin production

A

Dihydrobioptrein reductase deficiency

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9
Q

Tyrosinemias types

A

Type1 hepatorenal

Type2 oculocutaneous tyrosinemia

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10
Q

Type 1 hepatorenal

Deficiency

A

Fumarylacetoacetate hydrolase

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11
Q

Type 1 hepatorenal

Accumulation of

A

Fumarylacetoacetate

Maleyacetate

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12
Q

Type 1 hepatorenal

Result

A

DNA alkylation
Tumorigenesis
Rickets

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13
Q

Type 2 oculocutaneous tyrosinemia

Aka

A

Richner-hanhart syndrome

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14
Q

Type 2 oculocutaneous tyrosinemia

Deficiency

A

Tyrosine aminotransferase

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15
Q

Type 2 oculocutaneous tyrosinemia

Accumulation of

A

Tyrosine

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16
Q

Type 2 oculocutaneous tyrosinemia

Result

A

Eye lesion

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17
Q

Hyperphenylalanine type 1

Aka and defect

A

Classical PKU

Phenylalanine hydroxylase

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18
Q

Hyperphenylalaninemia 2 and 3

Defect

A

Dihydrobiopterin reductase

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19
Q

Hyperphenylalaninemia 4&5

Defect

A

Dihydrobiopterin biosynthesis

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20
Q

Hynurenine-anthranilate pathway

Major products

A

Serotonin and melatonin

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21
Q

Serotonin

A

Vasoconstrictor

Contraction of smooth muscle

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22
Q

Melatonin

A

Sleep inducing substance

Regulation of circadian rhythm

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23
Q

Location of synthesis of melatonin

A

Pineal gland and retina

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24
Hartnup disease Defect
Intestinal and renal transport of neutral amino acid
25
Hartnup disease Manifestation
Pellagra like | Tryptophan cant be converted to niacin
26
Blue diaper syndrome Manifestation
Indicanuria Hypercalcemia Nephrocalcinosis
27
Carcinoid syndrome Manifestation
``` Cyanotic flushing Watery stools Brochoconstriction Hypotension Edema ```
28
Carcinoid syndrome resulting to oversecretion of
Serotonin
29
Carcinoid syndrome diagnosis
Measure urinary 5-HIAA levels
30
Needed to convert N- formiminoglutamate (FIGLU) to glutamic acid
Tetrahydrofolic acid (vit B9)
31
Test for folic acid deficiency
FIGLU excretion test
32
Histidinemia Def
Histidase
33
Histidinemia Diagnosis
Skin biopsy | Urocanic acid in sweat
34
Histidinemia Manifestation
Seizures
35
Maple syrup urine disease Aka
Branched chain ketonuria
36
Maple syrup urine disease Defect
A keto acid decarboxylase
37
Maple syrup urine disease Accumulation of
Valine Isoleucine Leucine
38
Isovaleric academia For
Leucine only
39
Isovaleric academia Deficient
Isovaleryl CoA dehydrogenase
40
Lysine Production of
Saccharopine
41
Hyperlysinemia Deficient
Saccharone dehydrogenase
42
Hyperlysinemia Accumulation of
Lysine
43
Hyperlysinemia Action
Inhibits arginase
44
Hyperlysinemia End result
Increase ammonia level
45
Threonine pathways Threonine to a ketobutyric acid
Enzyme threonine dehydratase | End product glucose
46
Threonine pathways Threonine to glycine+acetaldehyde
Enzyme : threonine aldolase | End product : acetly CoA
47
Threonine pathways Threonine to aminoacetone
Reaction : dehydrogenation and decarboxylation End product : pyruvate
48
Methionine derivatives
``` Polyamines Carnitine Epinephrine Creatine phosphate Choline ```
49
Methionine Growth factors Stabilize intact cells Hypotensive agent
Polyamines ( spermidine and spermine)
50
Methionine Important for beta oxidation of fatty acids
Carnitine
51
Homocystinuria Deficiency
Cystathionine synthetase
52
Homocystinuria Accumulation of
Homocysteine
53
Homocystinuria Degrades
Collagen Elastin Proteoglycans
54
Homocystinuria Manifestation
Ocular abnormalities Osteoporosis Skeletal abnormalities Mental retardation
55
Cystathionuria Deficiency
Cystathionase
56
Cystathionuria Accumulation of
Cystathionine
57
Cysteine Derivatives
Coenzyme A | Taurine
58
Cystinuria Aka
Cysteine lysinuria
59
Cystinuria Accumulation of
Cystine
60
Cystinuria Formation of
Calculi
61
Cystinosis Aka
Cystine storage disease
62
Cystinosis Major effect
Impaired lysosomal function
63
Cystinosis Formation of
Cystine crystals
64
Glycine cleavage system Enzyme
Glycine syhtetase
65
Glycine conversion to serine
Serine hydroxymethyltransferase
66
Glycine oxidative deamination
Enzyme : glycine oxidase | Product : glyoxylic acid
67
Non ketotic hyperglycemia Deficiency
Glycine cleavage system
68
Non ketotic hyperglycemia Manifestation
Mental retardation
69
Glycine + succinyl CoA
Heme synthesis
70
Carbon 4&5 and nitrogen 7
Purine synthesis
71
Glycine + cholic acid
Glycocholic acid
72
Glycine + benzoic acid
Hippuric acid

Biochem 2nd sem (2 decks)

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