Topnotch Flashcards
(204 cards)
1
Q
Increase permeability of the inner mitrochondrial membrane to protons, thus proton gradient is lost without passing through the ATPase complex
A
Uncouplers
2
Q
examples of uncouplers
A
2,4 dinitrophenol Aspirin
3
Q
the only nonprotein part of ETC
A
Coenzyme Q
4
Q
property of water where it decreases the force of attraction between the charged and polar species
A
High dielectric constant
5
Q
Most important extracellular buffer
A
bicarbonate
6
Q
Most important intracellular buffer
A
proteins
7
Q
ETC part which is also part of Krebs
A
FADH Complex II
8
Q
Mobile part of ETC
A
Cytochome c
9
Q
Mitochondrial disease all complexes
A
Fatal Infantile Mitochondrial Myopathy
10
Q
Mitochondrial disease: Complex I
A
MELAS Mitochondrial encephalomyopathy, lactic acidosis, stroke like episodes
11
Q
Mitochondrial disease : Complex II
A
Kearns Sayre Syndrome
12
Q
Mitochondrial disease : Complex III
A
Leber’s Hereditary Optic Neuropathy
13
Q
Mitochondrial disease : Complex IV
A
Leigh’s Disease Ragged Red Muscle fiber disease
14
Q
Monosaccharide found in cardiac tissue
A
lyxose
15
Q
Component sucrose
A
Glucose + Fructose
16
Q
Glucose + Galactose
A
lactose
17
Q
3-10 sugars
A
oligosacchararides
18
Q
Compunds that differ in configuration in only one carbon
A
epimers
19
Q
mirror images of each other
A
enantiomers
20
Q
phenomenon that sugars convertible in alpha and beta form
A
mutarotation: anomers
21
Q
epimer of glucose at C4
A
galactose
22
Q
epimer of glucose at C2
A
mannose
23
Q
L and D forms of sugars
A
enantiomers
24
Q
Transported by SGLT in the intestines
A
Glucose and galactose
25
where is GLUT 2 found in the intestines?
basement membrane
26
transporter for fructose (facilitated transfusion) in intestinal brush border
GLUT 5
27
Where GLUT 1 is found
erythrocytes, brain, kidney, colon, placenta
28
GLUT 4 location
heart skeletak muscle adipose tissue
29
Where does it occur? Glycolysis
In the cytiplasm of all cells
30
Rate limiting step glycolysis
Phosphofuctokinase 1
31
3 irreversible and reulated stes in glycolysis
Phosphorylation of glucose Phosphorylation of fructose 6 phosphate formation of pyruvate
32
inhibitor of PFK 1
ATP citrate
33
Poison inhibits pyruvate dehydrogenase by binding to lipoic acid
Arsenic
34
Most common enzyme deficiency in glycolysis
PK
35
Krebs cycle occurs in the Mitochondrial matrix except
Succinate dehydrogenase
36
Rx pyruvate DH deficiency
ketogenic diet
37
Co-enzymes of Pyruvate dehydrogenase
Thiamine pyrophosphate FAD NAD Coenzyme A Lipoic Acid
38
ATP yield of glycolysis aerobic through malate shutte
8 ^ if aspartate
39
anaerobic glycolysis occurs where
RBCs lens cornea of eye kidney medulla testes WBCs
40
enzyme that catalyzes rate limiting enzyme of krebs
Isocitrate dehydrogenase
41
Enzyme in Krebs inhibited by arsenite
alpha ketoglutarate dehydrogenase
42
Enzyme in Krebs inhibited by rat poison (fluroacetate)
aconitase
43
This enzyme bypasses the first irreversible test of glycolysis
Glucose-6-phosphatase
44
Who will have higher 2,3 BPG levels, the manila girl or the baguio girl?
The baguio girl because 2,3 BPG levels will reduce hemoglobin affinity for O2
45
products of pyruvate DH complex
NADH Acetyl CoA ATP
46
enzyme that catalyzes the rate limiting step of gluconeogenesis
F 1, 6 bisphosphatase
47
Enzyme in cori's cycle found in liver that muscle lacks
Lactate dehydrogenase (catalyzes Lactate -\> Glucose)
48
Energy expense in Cori's cycle
4 ATPs
49
This gluconeogenesis enzyme bypasses pyruvate kinase
pyruvate carboxylase PEP carboxykinase
50
This gluconeogenesis enzyme bypasses PFK-1
F 1, 6 bisphosphatase
51
enzyme in gluconeogenesis requiring GTP
PEP carboxykinase
52
inhibits acetaldehyde DH
disulfiram
53
explain alcohol metabolism and consequences
high amounts of cytoplasmic NADH favors pyr to lactate oxaa to malate (decrease in gluconeogenesis) DHAP to G3P
http://commons.wikimedia.org/wiki/File:Benzyl\_Alcohol\_Metabolism\_Scheme.png
54
Where is glycogen stored?
100 g in liver (6% of liver) 400 g in muscle (1% of muscle)
55
Where does glycogenesis occur
liver and muscle (cytosol)
56
Enzyme catalyzing rate limiting step of glycogenesis
glycogen synthase
57
Where does glycogenolysis occur
lysosome of liver and muscle
58
Enzyme which catalyzes rate limiting step of glycogenolysis
glycogen phosphorylase
59
overview of glycogen metabolism
60
enzymes of the debranching enzyme
a(1-\>4) -\> a(1-\>4) glucan transferase
61
muscle cramps myoglobinuria
Mc Ardles
62
Deficiency in glucose 6 phosphatase
Von Gierke
63
deficiency in acid maltase, resulting in cardiomegaly and heart failure
Pompe's
64
Deficiency in debranching and branching enzyme
Cori's and andersen's respectively
65
* muscle cramps
* myoglobinuria
* hemolytic anemia
Tarui's (Type VII)
66
enzyme deficient in essential fructosuria
fructokinase
67
enzyme deficient in fructose intolerance
aldolase B
68
enzyme deficient in classic galactosemia
gal-1-P-uridyltransferase
69
Galactosemia is a CI to breastfeeding
:)
70
Fructose metabolism overview
71
mannose is an impt component of
glycoproteins
72
enzyme catalyzes glucose to sorbitol
aldose reductase
73
where aldose reductase is found
lens retina schwann cells liver kidney placenta
74
catalyzes sorbitol --\> fructose, and where is this found
sorbitol dehydrogenase only in SEMINAL VESICLES
75
enzyme catalyzes the rate limiting step of pentose phosphate pathway
glucose-6-phosphate DH
76
pathway for production of NADPH
PPP
77
where does PPP/ HMP occur
cytoplasm
78
where is NADPH needed
FA and steroid biosynthesis reduction of glutathione cytochrome p450 WBC respiratory burst nucleotide synthesis
79
What are the substrates?
glu 6 P
80
Overview of HMP 2 phases of HMP
**oxidative-** Glu 6 P DH
**nonoxidative**- transketolase (requires thiamine)
81
can be used to diagnose thiamine deficiency, cos it is its cofactor
RBC transketolase activity
82
required by glutatione peroxidase
selenium
83
enzyme sequesters H2O2 to H2O
glutathione peroxidase
84
def in NADPH oxidase
CGD
85
no. of carbons palmitic acid
16
86
2 essential FAs
linoleic acid omega 6 linolenic acd omega 3
87
arachidonic acid becomes essential if deficient
omega 6
88
FAs must be activated first before used in metabolism. what is the enzyme in activation of FA?
fatty acyl CoA synthetase
89
co-factor of fatty acyl CoA synthetase
patothenic acid (source of coenzymeA)
90
FAs must be activated first before used in metabolism. # of ATPs consumed in activation
2 ATPs
91
Rate limiting step of fatty acid synthesis
acetyl CoA carboxylase
92
where does FA synthesis occur
Cytosol liver and mammary glands minor: adipose tissue
93
3 steps FA synthesis
1. Synthesis of AcetylCoA (citrate shuttle ride)
2. Acetyl CoA carboxylated to malonyl CoA\*
3. Assembly of palmitate FATTY ACID SYNTHASE CodeRed D Red \*rate limiting
94
Sum up FA synthesis
1 AcetylCoA + 7Malonyl CoA + 14 NADPH
yields
1 palmitate + 7 Co2 + 6 H20 + 14 NADP
95
activates lipogenesis
citrate insulin
96
why are linoleic and linolenic acid assential
they have double bonds that exceed the 9th carbon
97
main storage forms of FAs
TAGs
98
Synthesis of TAGs
Glycerol 3 phosphate + 3 Fatty acyl CoA ---\> Tg
99
enzymes in TAG synthesis
* gly 3 P DH*
* glycerol kinase*
100
hydrolyzes TAGS yielding free FAs and glycerol
**Hormone sensitive lipase**,
only acting in C1 and C3 TAG -\> 2 free FA + 2 monoacylglycerol
101
where B oxidation occurs
mitochondria
102
products of B oxidation
8 acetyl CoA + 7 FADH2 + 7 NADH
103
enzyme that catalyzes rate limiting step in B oxidation
carnithine acyl transferase carnithine (palmitoyl) transferase
104
steps in B oxidation
Oxidation Hydration Oxidation Thiolysis
105
summary B oxidation
1 palmitate + 7 FAD + 7 NAD --\>
8 acetyl CoA + 7 FADH + 7 NADH
106
if FA had odd number
acetyl CoAs + propionyl CoA
107
oxidizes very long chain fatty acids
peroxisomes
108
NET ATPs from B oxidation
129 APT
109
The -2 ATP in the gross ATP yield in B oxidation is from
activation of palmitate
110
Deficiency of this can manifest as SIDS
Medium chain FA
111
caused by eting unripe fruit of akee tree which contains HYPOGLYCIN
Jamaican vomiting sickness
112
Inherited absence of peroxisomes in all tissues cerebrohepatorenal syndrome
zellweger's syndrome
113
defect in peroxysomal actibation of VLCFAs leading to its accumulation in blood and tissues
x-linked adrenoleukodystrophy
114
where are ketone bodies utilized
* brain
* renal cortex
* skeletal muscle
115
Rate limiting step enzyme of ketone body synthesis
HMG CoA synthase
116
substrate of ketone body synthesis
acetyl-CoA
117
Lack of this enzyme is reason why liver cannot use ketone bodies for fuel
THIOPHORASE succinyl CoA acetoacetyl CoA transferase
118
Nitroprusside test is a test for
ketones does not detect B hydroxybutyrate
119
of carbons in cholesterol
27
120
Rate limiting step enzyme of cholesterol synthesis
HMG CoA reductase (HMGCoA --\> mevalonate)
121
where does cholesterol synthesis occur
all cells sER
122
substrates cholesterol synthesis
acetyl CoA NADPH ATP
123
products cholesterol synthesis
lanosterol -\> cholesterol
124
competitive inhibitors of HMG CoA synthetase
statins
125
intestinal bacteria can reduce cholesterol to
coprostanol cholestanol
126
Rate limiting enzyme of bile acid synthesis
cholesterol 7 a hydroxylase
127
primary bile acids conjugated with ?
glycine and taurine to the secondary deoxycholic acid lithocholic acid
128
mother hormone steroid
pregnenolone
129
Rate limiting enzyme of steroid hormone synthesis
desmolase
130
Blocker of steroid hormone synthesis
aminogluthetimide
131
where steroid hormone synthesis occurs
SER of adrenal cortex ovaries and testes placenta
132
Overview steroid hormone synthesis
:) p 25 topnotch
133
apoprotein in chylomicrons
Apo B48
134
apoprotein in VLDL
Apo B-100
135
apoprotein in in HDL
Apo A1
136
apoprotein in the liver for uptake
Apo E
137
lipoprotein activates LCAt
found in HDL!! Apo A1
138
intestinal malabsorption, deficiency in apo B48 and B100, hence no chylomicron no VLDL
Abetalipoproteinemia
139
absence of Apo A1 hence no HDL
Familial a lipoprotein deficiency
140
most abundant phospholipid
phosphatidylcholine
141
phospholipid plays a role in apoptosis
phosphatidylserine
142
the only antigenic phospholipid found only in mitochondria
cardiolipin
143
decrease in ___ causes mitochondrial dysfunction in aging and heart failure
cardiolipin
144
sphingosine + FA
ceramide
145
ceramide + glu or gal
cerebroside
146
ceramide + oligosaccharide
globoside
147
ceramide + N acetyl neuramic acid
ganglioside
148
ceramide + sulfated galactose
sulfatides
149
lipid storage diseases, abnormal lipid DEGRADATION in lysosomes however synthesis is normal
sphingolipidoses
150
enzyme deficient in Tay Sach's disease
Hexosaminidase A
151
enzyme deficient in Fabry's disease
a- Galactosidase
152
enzyme deficient in Farber's disease
Ceramidase
153
Deficiency in B galactosidase
Krabbe's disease
154
Deficiency in B- glucosidase, hepatosplenomegaly erosion of long bones normal mentality
Gaucher's
155
deficient in Niemann Pick disease
sphingomyelinase
156
sphingolipidoses with triad of 1. skin rash 2. hoarseness 3. bone malformatin
Farber's disease
157
sphingolipidoses with the 3 Rs
* recessive x linked
* rash
* renal failure
Fabry's disease
158
All sphingolipidoses are autosomal except for ___ which is
fabry's disease x linked recessive
159
dietary precursor of eicosanoids
linoleic acid linoleic acid --\> arachidonic acid --\> :)
160
amino acid used in the first step of heme synthesis
GLYCINE glycine + succinyl CoA --\> d-ALA
161
amino acid used in purine synthesis
glycine
162
amino acid which carries nitrogen from peripheral tissue to liver
alanine
163
BCAAs
VAL
164
deficiency in branched chain a-ketoacid dehydrogenase
MSUD
165
amino acid with longest side chain
Trp
166
precursor of homocysteine
methionine
167
the imino acid
proline
168
found at phosphorylation site of enzyme modification
serine
169
where does O linked glycosylation occur
Golgi apparatus
170
where does N linked glycosylation occur
ER
171
amino acid for O linked glycosylation
serine threonine
172
amino acid for N linked glycosylation
asparagine
173
precursor for GABA
glutamate
174
test for folic acid deficiency ; as they excrete huge amounts of this in the urine after harge doses of Histidine
N-FIGlu test
175
part of aminoacid that accepts protons
amino group
176
part of amino acid that donates protons
carboxylic acid
177
Conditionally essential amino acids
Arginine Histidine
178
form of bonding in peptide bond
covalent
179
configuration of all amino acids in proteins
L config
180
reagent to remove C terminal amino acid
hydrazine carboxypeptidase
181
reagent to remove N terminal amino acid
sanger's reagent edman's reagent phenylisothiocyanate
182
amino acids per turn of spiral in alpha helix
3.6 aa
183
combinations of adjacent secondary structures such as
B-a-B unit, greek key, B meander
supersecondary structures
184
stabilizes the tertiary structures
* disulfide bonds
* hydrophobic reactons
* H bonds
* ionic bonds
185
functional and three dimensional structural units of a polypeptide
domains
186
Heme is a complex of ______ and \_\_\_\_\_
protoporphyrin IX ferrous iron
187
what is the major transporter of carbon dioxide in the blood
bicarbonate 75% hemoglobin (minor)
188
T (taut) form has low or high oxygen affinity?
low affinity
189
increase in protons shifts the equilibrium to the right whereas an increase in pO2 shifts the equilibrium to the left
Bohr effect
190
stabilizes the T structure of hemoglobin by creating more salt bridges
2,3 bisphosphoglycerate
191
antidote methemogobinemia
methylene blye
192
HbA1C, glucose glycosylates?
e-amino group of lysine
193
Diagnosed by osmotic fragility test
Hereditary spherocytosis
194
glu ---\> lysine substitution of Hb
Hb C disease
195
inadequate synthesis of alpha chains of hemoglobin
alpha thalassemia
196
Amino acids found in collagen
Glycine and Proline Gly-X-Y X= proline Y- hydroxyproline or hydroxylysine
197
Collagen in skin and blood vessels
Type III
198
Wound healing collagen
Type II to Type I
199
Brittle bone syndrome
Osteogenesis Imperfecta
200
Dietary deficiency of copper required by lysyl oxidase, impt in crosslinking in collagen
Menke's syndrome
201
Defect in Type VII collagen, characterized by skin breaks and blisters
Epidermolysis Bullosa
202
FIBRILLIN scaphoid linked by DESMOSINE
elastin
203
dolichostenomelia ectopia lentis
Marfan's syndrome
204
at risk for berry aneurisms
ehler's danlose syndrome
