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Biochem > Exam III > Flashcards

Exam III Flashcards

1
Q

Hunter Syndrome, MPS II

A

Iduronate Sulfatase

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2
Q

Sly Syndrome, MPS IV

A

b-glucuronidase

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3
Q

I-Cell

A

Mannose phosphorylation, lysosomal acid hydrolyase deficient, accumulation glycolipids and lysosomes

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4
Q

Hurler Syndrome, MPS I

A

alpha-L-iduronidase

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5
Q

Fabry Disease

A

a-Galactosidase

inability to sweat, kidney and heat failure

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6
Q

Niemann Pick

A

sphingomyelinase

hepatosplenomegaly

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7
Q

Tay-Sachs

A

hexosaminidase A

neurodegeneration, cherry red macula

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8
Q

Gaucher Disease

A

glucocerebrosidase

most prevelant, hepatosplenomegaly

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9
Q

Statins

A

competitive inhibitory HMG-CoA reductase

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10
Q

Smith Lemli-Opitz Syndrome

A

mutation DHCR7

low levels serum and tissue cholesterol

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11
Q

Hyperlipidemia Type I

A

Lipoprotein lipase deficiency
Apolipoprotein C2
milky serum, accumulation chylomicrons

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12
Q

Hyperlipidemia Type IIa

A

LDL receptors

hypercholesterolemia

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13
Q

Hyperlipidemia Type III

A

ApoE2 low affinity

lipid deposits in palmar creases

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14
Q

Hyperlipidemia Type IV

A

VLDL overproduction

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15
Q

Hyperlipidemia Type V

A

VLDL overproduction

Lipoprotein lipase deficiency

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16
Q

Tangier Disease

A

defective ABCA1 transporters
cholesterol accumulates inside cell
enlarged orange/yellow tonsils

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17
Q

LCAT Deficiency

A

LCAT
inability to esterify cholesterol
diffuse corneal opacities: Fish eye

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18
Q

MCAD

A

MCAD

children hypoglycemia

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19
Q

Methylmalonyl CoA mutase deficiency

A

methylmalonly CoA mutase

metabolic acidosis

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20
Q

Cushing’s Syndrome

A

excess cortisol

pituitary ACTH

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21
Q

Addison’s Disease

A

cortisol and aldosterone deficiency

hypoglycemia

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22
Q

Conn’s Syndrome

A

hyperaldosteronism

metabolic alkylosis

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23
Q

3beta-HSD

A

no synthesis corticosteroids or sex steroids

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24
Q

17alpha-Hydroxylase Deficiency

A

no cortisol or sex steroids

aldosterone

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25
Q

11beta-Hydroxylase Deficiency

A

no cortisol or aldosterone

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26
Q

21-hydroxylase Deficiency

A

no corticosteroids: MC, GC

enhanced androgen

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27
Q

Morning After Pill

A

progesterone antagonist

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28
Q

Osteoporosis postmenopausal

A

Estradiol E2 inhibits osteoclast activation

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29
Q

Hormone Replacement Therapy

A

estrogen + progestin

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30
Q

Cystinuria

A

defect reabsorption COAL: cystine, ornithine, arginine, lysine

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31
Q

Cystic Fibrosis

A

pancreatitis

lung and respiratory infections

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32
Q

Celiac Disease

A

inflammation small intestine

T-cell response to gluten

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33
Q

Hartnup Disorder

A

cannot absorb nonpolar amino acids

typtophan –> serotonin, melatonin, niacin

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34
Q

Glutamate and Ischemia induced Brain Damage

A

blood vessels in brain blocked

glutamate released –> apoptosis cascade

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35
Q

Hyperammonemia

A

high ammonia is neurotoxic

Glu elevated

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36
Q

Urea Cycle Genetic Deficiency

A

Ornithine Transcarbamoylase - OTC

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37
Q

Buphenyl

A

phenylbutarate

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38
Q

Cabalu

A

CPS-1

N-acetylglutamate structural analog

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39
Q

Ammonul

A

benzoate –> glycine, phenylacetate –> binds nitrogen –> excreted

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40
Q

BUN

A

Blood Urea Nitrogen

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41
Q

Type I CDG

A

defects oligosaccharide to protein

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42
Q

Type II CDG

A

defect trimming or processing sugars

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43
Q

Disorders Carnitine Shuttle

A

mitochondria

nutritional - vegans

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44
Q

Secondary Carnitine Deficiency

A

Acyl carnitine with CPT-2 or CACT

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45
Q

Jamaican Vomiting Sickness

A

Hypoglycin A
sequester carnitine and CoA
inhibits beta-oxidation FA

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46
Q

Fibrates

A

GEmfibrozil, Fenofibrate
Decreases TAG levels
PPAR-alpha agonist –> increase lipoprotein lipase

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47
Q

Bile Acid Resins

A

Cole-drugs

bind bile acid to prevent reabsorption

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48
Q

Alprostadil

A

PGE analog, vasodilates

49
Q

Misoprostadil

A

PGE analog

stomach lining with NSAIDs

50
Q

Latanoprost

A

PGF2a analog

treats glaucoma

51
Q

Epoprostenol

A

PGI2 analog

treats pulmonary hypertension

52
Q

Dinoprostone

A

PGE analog

induces labor, aborts baby

53
Q

NSAIDS - ibuprofen, motrin, acetaminophem

A

non-selective COX 1, 2 inhibitors

54
Q

Aspirin

A

irreversible COX 1, 2 inhibitor

low dose to prevent clotting

55
Q

Zileuton

A

Lipoxygenase blocker

decreases leukotriene synthesis

56
Q

Zafir/monte - lukast

A

LTC4, LTD4 receptor antagonist

decreases leukotriene action

57
Q

Celecoxib

A

selective COX2 inhibitor

stops inflammatory pain

58
Q

Rate limiting step of Urea Cycle

A

Carbomoyl phosphate

59
Q

Rate limiting enzyme Urea Cycle

A

CPS1

carbomoyl phosphate synthase 1

60
Q

Ammonia Sources

A
  1. transamination/deamination
  2. glutamine –> glutamate
  3. Urea
  4. MAO
  5. Nucleic Acid –> adenosine deaminase
61
Q

Ammonia Transport

A

Glutamine
Alanine
Ammonia - intestines

62
Q

Induction Urea Cycle

A

protein, starvation, glucagon

63
Q

Nitrogen sources in Urea Cycle

A

CPS1

Aspartate

64
Q

Products Urea Cycle

A

Urea + fumarate

65
Q

Rate limiting step steroid hormones

A

StAR

66
Q

Steps under hormonal control in steroid hormone synthesis

A

StAR, p450, desmolase

67
Q

Regulation steroid hormone synthesis

A

pituitary hormones

ACTH, LH, FSH

68
Q

Steroid hormonal feedback regulation

A

adrenal steroidogenesis

gonadal steroidogenesis

69
Q

Mineralcorticoids

A

Zona Glomerulos

Aldosterone

70
Q

Glucocorticoids

A

Zona Fasticulata

Cortisol

71
Q

Adrenal Androgens

A

Zona Reticularis

DHEA

72
Q

Catecholamines

A

Medulla

Ep, NE

73
Q

Glucocorticoids

A

Cortisol

muscle, liver, adipose, kidney, colon, parotid

74
Q

Mineralocorticoids

A

Aldosterone

Kidney, colon, parotid

75
Q

Androgen

A

DHEA, testosterone

reproductive organs, muscle

76
Q

Estrogen

A

Estadiol, Estrone, Estriol

Reproductive organs

77
Q

Progestin

A

Progesterone

Uterus

78
Q

Adrenal

A

Glomerulosa, Fasiculata, Reticularis

Aldosterone, Cortisol, DHEA

79
Q

Testis

A

Leydig cells

Testosterone

80
Q

Ovary

A

Follicle, stroma, corpus luteum

Estradiol, Progesterone

81
Q

Placenta

A

Progesterone, Estradiol, Estriol

82
Q

Peripheral Tissue

A

Adipose, brain

Estrone, Estradiol

83
Q

Oxidative Deamination

A

transamination, deamination

84
Q

Transamination: pyruvic acid

A

alanine

85
Q

Transamination: oxaloacetate

A

aspartic acid

86
Q

Transamination: alpha ketoglutarate

A

glutamic acid

87
Q

ALT

A

alanine transaminase

Liver

88
Q

AST

A

aspartate transamination
skeletal, cardiac, liver
Vitamin B6

89
Q

Vitamin B6

A

pyridoxal phosphate

nitrogen and protein metabolism

90
Q

Vitamin B6 Reactions

A 
DOPA --> dopamine
Met --> Cys
vitamin --> cofactor
niacin --> NADPH
riboflavin --> FADH2
91
Q

Liver Function Test: ALT

A

8 - 37 IU/L

92
Q

Liver Function Test: AST

A

10 - 34 IU/L

93
Q

alpha KG –> glutamate

A

NADPH, brain

94
Q

glutamate –> alpha KG

A

NAD+, liver

95
Q

D-amino acid oxidase, DAO

A

DAO –> peroxisomal FAD –> alpha KG
oxidizes D-serine in the brain –> pyroxide
schizophrenia

96
Q

Most abundant neurotransmitter

A

Glutamate

97
Q

Glutamate Ischemia

A

no neuronal O2 –> increase glutamate –> NMDA binds –> Na+ and H2O influx –> neurons swell and apoptosis

98
Q

Essential amino acid

A

PVT TIM HALL
Phe, Val, Thr
Trp, Ile, Met
His, Arg, Leu, Lys

99
Q

Protein Storage

A

Muscle - 17% body weight

100
Q

Source amino acids

A

dietary protein
body protein
non-essential a acids

101
Q

Fat Soluble Vitamins

A

A, D, E, K

102
Q

Positive nitrogen balance

A

input > output

pregnancy, growth

103
Q

Negative nitrogen balance

A

input < output

fasting, fever, burns

104
Q

Protein turn-over

A

N-terminus

105
Q

Protein half-life

A

PEST

proline, glutamic acid, serine, theroine

106
Q

Protein Degradation

A

Ubiquitin-proteosome

Lysosomal Degradation

107
Q

Ubiquitin-proteosome

A

endogenous, nuclear, cytosolic

108
Q

Lysosomal Degradation

A

extracellular

109
Q

Stomach Digestion

A

HCl, parietal cells

110
Q

Pepsinogen –> pepsin

A

Chief cells

111
Q

Trypsin

A

basic amino acids

112
Q

Chymotrypsin

A

bulky amino acids

113
Q

Elastase

A

small amino acids

114
Q

Carboxypeptidase

A

carboxyl terminus

115
Q

Mechanism enterocyte absorption

A

Na/K ATPase

116
Q

Mechanism di-tri-peptides

A

PEPT-1

117
Q

Glycoprotein

A

primarily protein

oligosaccharides

118
Q

Proteoglycan

A

primary carbohydrate
negatively charged
GAG

Biochem (4 decks)

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