Block 3 Biochem Flashcards
(126 cards)
1
Q
what is the committed reaction in steroid hormone sythesis?
A
cholesterol to pregnenolone
2
Q
3 hydroxylations of progesterone makes
A
cortisol
3
Q
2 hydroxylations and an oxidation of progesterone makes
A
aldosterone
4
Q
3 beta hydroxysteroid dehyd. deficiency results in
A
pregnenolone buildup, marked salt excretion. also female genitalia
5
Q
17 alpha hydroxylase deficiency results in
A
no sex hormones or cortisol. more aldosterone made
6
Q
21 alpha hydroxylase deficiency-
A
no gluco or mineralocorticoids made
7
Q
Angiotensin activates what protein kinase to stimulate aldosterone?
A
PKC
8
Q
pepsinogen is secreted by _____
A
chief cells
9
Q
pepsin is a ______ protease
A
aspartate
10
Q
HCl is secreted by _______
A
parietal cells
11
Q
signals the release of bicarb from the pancreas-
A
secretin
12
Q
what converts trypsinogen to trypsin?
A
enteropeptidase
13
Q
what activates most pancreatic zymogens?
A
trypsin
14
Q
serine proteases are what kind of peptidases?
A
endopeptidases
15
Q
trypsin cuts at what AA’s
A
C terminus arginines and lysines
16
Q
elastase cuts what kinds of AA’s
A
small ones (ala, gly, ser)
17
Q
are metallopeptidases endo or exo peptidases?
A
exo
18
Q
Defect in COAL transport can lead to
A
cystinuria
19
Q
hartnup disorder-
A
inability to reabsorb bulky AA’s
20
Q
what coenzyme do aminotransferases use?
A
PLP (B6) derivative
21
Q
which 2 aa’s do not undergo aminotransferase rxns
A
lysine and threonine
22
Q
where is DAO located?
A
in peroxisomes
23
Q
Cahill cycle-
A
alanine from muscle is transported to liver to be converted to pyruvate
24
Q
blood sugar conversion factor. 1 mM = __ mg/dL
A
18
25
list the 4 metabolic enzymes that are active while phosphorylated
hormone sensitive lipase, glycogen phosphorylase kinase, and glycogen phosphorylase, fructose bisphosphate phosphatase 2
26
binge drinking can drive OAA towards malate production, leading to
alcoholic ketoacidosis, as there is now excess acetyl CoA
27
after 3 weeks of fasting, muscle quits using ketone bodies for energy, only using FFA. This makes it easier for....
brain to use ketone bodies
28
during prolonged fasting, what provides 50% of glucose to muscle?
kidneys
29
which secretion of adipose tissue improves insulin sensitivity?
adiponectin
30
as we get fatter, what happens to adiponectin levels?
they decrease
31
how do FFA and TNF alpha promote insulin resistance?
they bind to serine and threonine residues on insulin receptors and make the tyrosine residues unavailable to do its thing
32
macrovascular complications begin with
hyperinsulinemia
33
microvascular complications begin with
impaired glucose tolerance
34
BMI =
weight in kg/height m^2 or weight lb/height^2 inches x 703
35
do non-diabetic obese people have hyperglycemia?
no
36
what inhibits the synthesis of THF in bacteria?
sulfonamide
37
Methotrexate:
anti cancer drug. stops all synthesis of THF
38
what enzyme does methotrexate target?
dihydrofolate reductase
39
how is excess folate a problem?
can mask B12 deficiency
40
B12 aka
cobalamin
41
B12 used for synthesis of
methionine
42
enzyme responsible for uptake of B12
intrinsic factor
43
1 kg = ___ lbs
2.2
44
ideally our calories should come from (%)
carbs
fats
protein
carbs 55%
fats 25%
protein 20%
45
men should get ___ g fiber
38
46
essential n-3 FA in humans
alpha linoleic acid
47
which vitamins prevent CHD and how?
B6,12, and 9. Lower homocysteine levels
48
saturated fatty acids ___ LDL and ___ HDL
increase
| no change
49
n-6 PUFA's ____ LDL and ____ HDL
decrease both
50
n-3 effect on LDL/HDL
none
51
B12 is used for _______ catabolism, in which it is a coenzyme for ________
odd chain fatty acid\
| methylmalonyl CoA mutase
52
deficiency of B12 =
pernicious anemia
53
B6 aka
pyridoxal phosphate
54
B6 is a cofactor for sythesis of:
heme and histamine
55
Beriberi disease associated with what vitamin?
Thiamine
56
wericke-korsakoff syndrome associated with what vitamin?
Thiamine
57
niacin is a precursor of
NADH and NADPH
58
Niacin deficiency =
pellagra
59
niacin inhibits _____ in adipose tissue
lipolysis
60
B2 aka
riboflavin
61
B2/riboflavin is a precursor for
FAD and FMN
62
Biotin is a coenzyme of _____ reactions
carboxylation
63
B5 aka
pantothenic acid
64
rhodopsin =
retinal + opsin
65
defect in cleavage of glycine. diet restrictions ineffective
nonketotic hyperglycinemia
66
cystathione synthase defect or B6 deficiency leading to accumulation of methionine. OR... a folic acid or B12 deficit, which would actually cause low levels of methionine
homocystinuria (looks like marfans)
67
most common urea cycle disorder
ornithine transcarbamoylase deficiency
68
ornithine transcarbamoylase deficiency would manifest as
respiratory alkalosis, hyperammonemia, elevated orotic acid
69
3 organic acidemias
propionic, methylmalonic and glutaric acidemia
70
organic acidemia-
defect leading to accumulation of intermediates of catabolism of macromolecules.
71
Pt has wide anion gap, ketonuria, and maybe hyperammonemia. what kind of disease?
organic acidemia
72
propionic acidemia-
biotin deficiency or enzyme deficient, accumulation of propionyl coa
73
methylmalonic acidemia-
enzyme or B12 deficiency, accumulation ofmethylmalonyl CoA
74
Hypoketotic/hypoglycemic patient has no ketones in urine. What do they have?
Medium chain Acyl CoA dehydrogenase defect
75
patient has hypoglycemia, low levels of ketones, and metabolic acidosis. What do they have?
Long chain acyl CoA dehydrogenase deficiency
76
cofactor for tyrosine hydroxylase
BH4
77
rate limiting step of catecholamine sythesis
tyrosine hydroxylase making DOPA
78
DOPA decarboxylase cofactor
PLP
79
dopamine--> norepi enzyme
dopamine beta hydroxylase
80
Norepi--> epi enzyme
phenylethanolamine-N-methyl-transferase
81
rate limiting step of 5-HT synthesis-
tryptophan-->5 hydroxytryptophan
82
rate limiting step of creatine synthesis
arginine+glycine-->guanidinoacetate+ornithine via amidinotransferase
83
H2 histamine receptor stimulates-
gastric secretions, smooth muscle relaxation
84
hereditary fructose intolerance ED
Aldolase B
85
Von Gierke Type 1 ED
G6 phosphatase
86
Von Gierke Type 2 ED
G6 translocase
87
Pompe disease ED
lysosomal alpha glucosidase
88
Cori disease ED
debranching enz.
89
McArdle disease ED
muscle glycogen phosphorylase
90
5 mucopolysaccharidoses-
hurler, hunter, sanfilipo, morquio, and maroteaux-lamy
91
hurler syndrome ED
alpha L iduronase
92
Hunter syndrome ED
iduronate sulfatase
93
sanfilipo syndrome ED
heparan sulfate degrading enzyme def.
94
Morquio synd. ED
keratin sulfate degrading enzyme def.
95
Maroteaux-Lamy ED
arylsulfatase B
96
Neimann Pick ED
sphingomyelinase
97
Tay Sachs ED
Hexosaminidase A
98
GM1 ganglioacidosis ED
beta galactosidase
99
Metachromatic leukodystrophy ED
arylsulfatase A
100
Alpha-mannosidosis ED
alpha D mannosidase
101
zellweger syndrome-
cant break down very long chain fatty acids
102
Smith-Lemli-Opitz syndrome-
deficient in 7 dehydrocholesterol reductase. low cholesterol in body. polydactyly
103
cherry red macula associated with
lysosomal storage diseases (glycolipidoses)
104
list the nonessential glucogenic amino acids
All the A's and G's. + CSHP
105
list the essential glucogenic AA's
HMTV (Hate MTV)
106
list the nonessential amino acid that is both gluco and ketogenic
Tyrosine
107
list the 3 essential gluco/ketogenic aa's
isoleucine, phenylalanine, and tryptophan (WIF)
108
list the 2 essential ketogenic aa's
Leucine, lysine (The L's)
109
are there nonessential ketogenic aa's
NO!
110
pro-->glu
proline oxidase
111
arg--> ornithine
arginase
112
OAA is synth. from what aa?
aspartate
113
pyruvate is synth from what aa?
alanine
114
ornithine--> glu
ornithine aminotransferase
115
his-->glu
histidase
116
ser-->pyruvate
serine dehydratase
117
sery-->gly
serine hydroxymethyl transferase
118
Thr-->succ CoA
threonine dehydratase
119
Phe-->fumarate
phenylalanine hydroxylase (uses BH4)
120
homocysteine-->cystathinone-->cysteine
cystathione beta synthase (req. B6)
121
proline is formed by cyclization of
glutamine
122
urocanic aciduria caused by deficiency in urocanase or
overactive histidase
123
_____ deficiency mimics PKU
BH4
124
homocystinuria ED
cystathione b sythase
125
Alkaptonuria ED
homogenistic acid oxidase
126
treatment for alkaptonuria-
inhibition of 4-hydroxyphenylpyruvate dioxygenase
