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Biochem > Exam III > Flashcards

Exam III Flashcards

(118 cards)

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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
11beta-Hydroxylase Deficiency
no cortisol or aldosterone
26
21-hydroxylase Deficiency
no corticosteroids: MC, GC | enhanced androgen
27
Morning After Pill
progesterone antagonist
28
Osteoporosis postmenopausal
Estradiol E2 inhibits osteoclast activation
29
Hormone Replacement Therapy
estrogen + progestin
30
Cystinuria
defect reabsorption COAL: cystine, ornithine, arginine, lysine
31
Cystic Fibrosis
pancreatitis | lung and respiratory infections
32
Celiac Disease
inflammation small intestine | T-cell response to gluten
33
Hartnup Disorder
cannot absorb nonpolar amino acids | typtophan --> serotonin, melatonin, niacin
34
Glutamate and Ischemia induced Brain Damage
blood vessels in brain blocked | glutamate released --> apoptosis cascade
35
Hyperammonemia
high ammonia is neurotoxic | Glu elevated
36
Urea Cycle Genetic Deficiency
Ornithine Transcarbamoylase - OTC
37
Buphenyl
phenylbutarate
38
Cabalu
CPS-1 | N-acetylglutamate structural analog
39
Ammonul
benzoate --> glycine, phenylacetate --> binds nitrogen --> excreted
40
BUN
Blood Urea Nitrogen
41
Type I CDG
defects oligosaccharide to protein
42
Type II CDG
defect trimming or processing sugars
43
Disorders Carnitine Shuttle
mitochondria | nutritional - vegans
44
Secondary Carnitine Deficiency
Acyl carnitine with CPT-2 or CACT
45
Jamaican Vomiting Sickness
Hypoglycin A sequester carnitine and CoA inhibits beta-oxidation FA
46
Fibrates
GEmfibrozil, Fenofibrate Decreases TAG levels PPAR-alpha agonist --> increase lipoprotein lipase
47
Bile Acid Resins
Cole-drugs | bind bile acid to prevent reabsorption
48
Alprostadil
PGE analog, vasodilates
49
Misoprostadil
PGE analog | stomach lining with NSAIDs
50
Latanoprost
PGF2a analog | treats glaucoma
51
Epoprostenol
PGI2 analog | treats pulmonary hypertension
52
Dinoprostone
PGE analog | induces labor, aborts baby
53
NSAIDS - ibuprofen, motrin, acetaminophem
non-selective COX 1, 2 inhibitors
54
Aspirin
irreversible COX 1, 2 inhibitor | low dose to prevent clotting
55
Zileuton
Lipoxygenase blocker | decreases leukotriene synthesis
56
Zafir/monte - lukast
LTC4, LTD4 receptor antagonist | decreases leukotriene action
57
Celecoxib
selective COX2 inhibitor | stops inflammatory pain
58
Rate limiting step of Urea Cycle
Carbomoyl phosphate
59
Rate limiting enzyme Urea Cycle
CPS1 | carbomoyl phosphate synthase 1
60
Ammonia Sources
1. transamination/deamination 2. glutamine --> glutamate 3. Urea 4. MAO 5. Nucleic Acid --> adenosine deaminase
61
Ammonia Transport
Glutamine Alanine Ammonia - intestines
62
Induction Urea Cycle
protein, starvation, glucagon
63
Nitrogen sources in Urea Cycle
CPS1 | Aspartate
64
Products Urea Cycle
Urea + fumarate
65
Rate limiting step steroid hormones
StAR
66
Steps under hormonal control in steroid hormone synthesis
StAR, p450, desmolase
67
Regulation steroid hormone synthesis
pituitary hormones | ACTH, LH, FSH
68
Steroid hormonal feedback regulation
adrenal steroidogenesis | gonadal steroidogenesis
69
Mineralcorticoids
Zona Glomerulos | Aldosterone
70
Glucocorticoids
Zona Fasticulata | Cortisol
71
Adrenal Androgens
Zona Reticularis | DHEA
72
Catecholamines
Medulla | Ep, NE
73
Glucocorticoids
Cortisol | muscle, liver, adipose, kidney, colon, parotid
74
Mineralocorticoids
Aldosterone | Kidney, colon, parotid
75
Androgen
DHEA, testosterone | reproductive organs, muscle
76
Estrogen
Estadiol, Estrone, Estriol | Reproductive organs
77
Progestin
Progesterone | Uterus
78
Adrenal
Glomerulosa, Fasiculata, Reticularis | Aldosterone, Cortisol, DHEA
79
Testis
Leydig cells | Testosterone
80
Ovary
Follicle, stroma, corpus luteum | Estradiol, Progesterone
81
Placenta
Progesterone, Estradiol, Estriol
82
Peripheral Tissue
Adipose, brain | Estrone, Estradiol
83
Oxidative Deamination
transamination, deamination
84
Transamination: pyruvic acid
alanine
85
Transamination: oxaloacetate
aspartic acid
86
Transamination: alpha ketoglutarate
glutamic acid
87
ALT
alanine transaminase | Liver
88
AST
aspartate transamination skeletal, cardiac, liver Vitamin B6
89
Vitamin B6
pyridoxal phosphate | nitrogen and protein metabolism
90
Vitamin B6 Reactions
``` DOPA --> dopamine Met --> Cys vitamin --> cofactor niacin --> NADPH riboflavin --> FADH2 ```
91
Liver Function Test: ALT
8 - 37 IU/L
92
Liver Function Test: AST
10 - 34 IU/L
93
alpha KG --> glutamate
NADPH, brain
94
glutamate --> alpha KG
NAD+, liver
95
D-amino acid oxidase, DAO
DAO --> peroxisomal FAD --> alpha KG oxidizes D-serine in the brain --> pyroxide schizophrenia
96
Most abundant neurotransmitter
Glutamate
97
Glutamate Ischemia
no neuronal O2 --> increase glutamate --> NMDA binds --> Na+ and H2O influx --> neurons swell and apoptosis
98
Essential amino acid
PVT TIM HALL Phe, Val, Thr Trp, Ile, Met His, Arg, Leu, Lys
99
Protein Storage
Muscle - 17% body weight
100
Source amino acids
dietary protein body protein non-essential a acids
101
Fat Soluble Vitamins
A, D, E, K
102
Positive nitrogen balance
input > output | pregnancy, growth
103
Negative nitrogen balance
input < output | fasting, fever, burns
104
Protein turn-over
N-terminus
105
Protein half-life
PEST | proline, glutamic acid, serine, theroine
106
Protein Degradation
Ubiquitin-proteosome | Lysosomal Degradation
107
Ubiquitin-proteosome
endogenous, nuclear, cytosolic
108
Lysosomal Degradation
extracellular
109
Stomach Digestion
HCl, parietal cells
110
Pepsinogen --> pepsin
Chief cells
111
Trypsin
basic amino acids
112
Chymotrypsin
bulky amino acids
113
Elastase
small amino acids
114
Carboxypeptidase
carboxyl terminus
115
Mechanism enterocyte absorption
Na/K ATPase
116
Mechanism di-tri-peptides
PEPT-1
117
Glycoprotein
primarily protein | oligosaccharides
118
Proteoglycan
primary carbohydrate negatively charged GAG

Biochem (4 decks)

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