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Z_Biochem > Topnotch > Flashcards

Topnotch Flashcards

1
Q

Increase permeability of the inner mitrochondrial membrane to protons, thus proton gradient is lost without passing through the ATPase complex

A

Uncouplers

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2
Q

examples of uncouplers

A

2,4 dinitrophenol Aspirin

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3
Q

the only nonprotein part of ETC

A

Coenzyme Q

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4
Q

property of water where it decreases the force of attraction between the charged and polar species

A

High dielectric constant

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5
Q

Most important extracellular buffer

A

bicarbonate

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6
Q

Most important intracellular buffer

A

proteins

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7
Q

ETC part which is also part of Krebs

A

FADH Complex II

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8
Q

Mobile part of ETC

A

Cytochome c

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9
Q

Mitochondrial disease all complexes

A

Fatal Infantile Mitochondrial Myopathy

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10
Q

Mitochondrial disease: Complex I

A

MELAS Mitochondrial encephalomyopathy, lactic acidosis, stroke like episodes

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11
Q

Mitochondrial disease : Complex II

A

Kearns Sayre Syndrome

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12
Q

Mitochondrial disease : Complex III

A

Leber’s Hereditary Optic Neuropathy

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13
Q

Mitochondrial disease : Complex IV

A

Leigh’s Disease Ragged Red Muscle fiber disease

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14
Q

Monosaccharide found in cardiac tissue

A

lyxose

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15
Q

Component sucrose

A

Glucose + Fructose

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16
Q

Glucose + Galactose

A

lactose

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17
Q

3-10 sugars

A

oligosacchararides

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18
Q

Compunds that differ in configuration in only one carbon

A

epimers

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19
Q

mirror images of each other

A

enantiomers

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20
Q

phenomenon that sugars convertible in alpha and beta form

A

mutarotation: anomers

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21
Q

epimer of glucose at C4

A

galactose

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22
Q

epimer of glucose at C2

A

mannose

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23
Q

L and D forms of sugars

A

enantiomers

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24
Q

Transported by SGLT in the intestines

A

Glucose and galactose

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25
Q

where is GLUT 2 found in the intestines?

A

basement membrane

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26
Q

transporter for fructose (facilitated transfusion) in intestinal brush border

A

GLUT 5

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27
Q

Where GLUT 1 is found

A

erythrocytes, brain, kidney, colon, placenta

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28
Q

GLUT 4 location

A

heart skeletak muscle adipose tissue

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29
Q

Where does it occur? Glycolysis

A

In the cytiplasm of all cells

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30
Q

Rate limiting step glycolysis

A

Phosphofuctokinase 1

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31
Q

3 irreversible and reulated stes in glycolysis

A

Phosphorylation of glucose Phosphorylation of fructose 6 phosphate formation of pyruvate

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32
Q

inhibitor of PFK 1

A

ATP citrate

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33
Q

Poison inhibits pyruvate dehydrogenase by binding to lipoic acid

A

Arsenic

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34
Q

Most common enzyme deficiency in glycolysis

A

PK

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35
Q

Krebs cycle occurs in the Mitochondrial matrix except

A

Succinate dehydrogenase

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36
Q

Rx pyruvate DH deficiency

A

ketogenic diet

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37
Q

Co-enzymes of Pyruvate dehydrogenase

A

Thiamine pyrophosphate FAD NAD Coenzyme A Lipoic Acid

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38
Q

ATP yield of glycolysis aerobic through malate shutte

A

8 ^ if aspartate

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39
Q

anaerobic glycolysis occurs where

A

RBCs lens cornea of eye kidney medulla testes WBCs

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40
Q

enzyme that catalyzes rate limiting enzyme of krebs

A

Isocitrate dehydrogenase

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41
Q

Enzyme in Krebs inhibited by arsenite

A

alpha ketoglutarate dehydrogenase

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42
Q

Enzyme in Krebs inhibited by rat poison (fluroacetate)

A

aconitase

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43
Q

This enzyme bypasses the first irreversible test of glycolysis

A

Glucose-6-phosphatase

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44
Q

Who will have higher 2,3 BPG levels, the manila girl or the baguio girl?

A

The baguio girl because 2,3 BPG levels will reduce hemoglobin affinity for O2

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45
Q

products of pyruvate DH complex

A

NADH Acetyl CoA ATP

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46
Q

enzyme that catalyzes the rate limiting step of gluconeogenesis

A

F 1, 6 bisphosphatase

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47
Q

Enzyme in cori’s cycle found in liver that muscle lacks

A

Lactate dehydrogenase (catalyzes Lactate -> Glucose)

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48
Q

Energy expense in Cori’s cycle

A

4 ATPs

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49
Q

This gluconeogenesis enzyme bypasses pyruvate kinase

A

pyruvate carboxylase PEP carboxykinase

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50
Q

This gluconeogenesis enzyme bypasses PFK-1

A

F 1, 6 bisphosphatase

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51
Q

enzyme in gluconeogenesis requiring GTP

A

PEP carboxykinase

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52
Q

inhibits acetaldehyde DH

A

disulfiram

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53
Q

explain alcohol metabolism and consequences

A

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

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54
Q

Where is glycogen stored?

A

100 g in liver (6% of liver) 400 g in muscle (1% of muscle)

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55
Q

Where does glycogenesis occur

A

liver and muscle (cytosol)

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56
Q

Enzyme catalyzing rate limiting step of glycogenesis

A

glycogen synthase

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57
Q

Where does glycogenolysis occur

A

lysosome of liver and muscle

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58
Q

Enzyme which catalyzes rate limiting step of glycogenolysis

A

glycogen phosphorylase

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59
Q

overview of glycogen metabolism

A
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60
Q

enzymes of the debranching enzyme

A

a(1->4) -> a(1->4) glucan transferase

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61
Q

muscle cramps myoglobinuria

A

Mc Ardles

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62
Q

Deficiency in glucose 6 phosphatase

A

Von Gierke

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63
Q

deficiency in acid maltase, resulting in cardiomegaly and heart failure

A

Pompe’s

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64
Q

Deficiency in debranching and branching enzyme

A

Cori’s and andersen’s respectively

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65
Q
  • muscle cramps
  • myoglobinuria
  • hemolytic anemia
A

Tarui’s (Type VII)

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66
Q

enzyme deficient in essential fructosuria

A

fructokinase

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67
Q

enzyme deficient in fructose intolerance

A

aldolase B

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68
Q

enzyme deficient in classic galactosemia

A

gal-1-P-uridyltransferase

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69
Q

Galactosemia is a CI to breastfeeding

A

:)

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70
Q

Fructose metabolism overview

A
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71
Q

mannose is an impt component of

A

glycoproteins

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72
Q

enzyme catalyzes glucose to sorbitol

A

aldose reductase

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73
Q

where aldose reductase is found

A

lens retina schwann cells liver kidney placenta

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74
Q

catalyzes sorbitol –> fructose, and where is this found

A

sorbitol dehydrogenase only in SEMINAL VESICLES

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75
Q

enzyme catalyzes the rate limiting step of pentose phosphate pathway

A

glucose-6-phosphate DH

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76
Q

pathway for production of NADPH

A

PPP

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77
Q

where does PPP/ HMP occur

A

cytoplasm

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78
Q

where is NADPH needed

A

FA and steroid biosynthesis reduction of glutathione cytochrome p450 WBC respiratory burst nucleotide synthesis

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79
Q

What are the substrates?

A

glu 6 P

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80
Q

Overview of HMP 2 phases of HMP

A

oxidative- Glu 6 P DH

nonoxidative- transketolase (requires thiamine)

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81
Q

can be used to diagnose thiamine deficiency, cos it is its cofactor

A

RBC transketolase activity

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82
Q

required by glutatione peroxidase

A

selenium

83
Q

enzyme sequesters H2O2 to H2O

A

glutathione peroxidase

84
Q

def in NADPH oxidase

A

CGD

85
Q

no. of carbons palmitic acid

A

16

86
Q

2 essential FAs

A

linoleic acid omega 6 linolenic acd omega 3

87
Q

arachidonic acid becomes essential if deficient

A

omega 6

88
Q

FAs must be activated first before used in metabolism. what is the enzyme in activation of FA?

A

fatty acyl CoA synthetase

89
Q

co-factor of fatty acyl CoA synthetase

A

patothenic acid (source of coenzymeA)

90
Q

FAs must be activated first before used in metabolism. # of ATPs consumed in activation

A

2 ATPs

91
Q

Rate limiting step of fatty acid synthesis

A

acetyl CoA carboxylase

92
Q

where does FA synthesis occur

A

Cytosol liver and mammary glands minor: adipose tissue

93
Q

3 steps FA synthesis

A
  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
Q

Sum up FA synthesis

A

1 AcetylCoA + 7Malonyl CoA + 14 NADPH

yields

1 palmitate + 7 Co2 + 6 H20 + 14 NADP

95
Q

activates lipogenesis

A

citrate insulin

96
Q

why are linoleic and linolenic acid assential

A

they have double bonds that exceed the 9th carbon

97
Q

main storage forms of FAs

A

TAGs

98
Q

Synthesis of TAGs

A

Glycerol 3 phosphate + 3 Fatty acyl CoA —> Tg

99
Q

enzymes in TAG synthesis

A
  • gly 3 P DH*
  • glycerol kinase*
100
Q

hydrolyzes TAGS yielding free FAs and glycerol

A

Hormone sensitive lipase,

only acting in C1 and C3 TAG -> 2 free FA + 2 monoacylglycerol

101
Q

where B oxidation occurs

A

mitochondria

102
Q

products of B oxidation

A

8 acetyl CoA + 7 FADH2 + 7 NADH

103
Q

enzyme that catalyzes rate limiting step in B oxidation

A

carnithine acyl transferase carnithine (palmitoyl) transferase

104
Q

steps in B oxidation

A

Oxidation Hydration Oxidation Thiolysis

105
Q

summary B oxidation

A

1 palmitate + 7 FAD + 7 NAD –>

8 acetyl CoA + 7 FADH + 7 NADH

106
Q

if FA had odd number

A

acetyl CoAs + propionyl CoA

107
Q

oxidizes very long chain fatty acids

A

peroxisomes

108
Q

NET ATPs from B oxidation

A

129 APT

109
Q

The -2 ATP in the gross ATP yield in B oxidation is from

A

activation of palmitate

110
Q

Deficiency of this can manifest as SIDS

A

Medium chain FA

111
Q

caused by eting unripe fruit of akee tree which contains HYPOGLYCIN

A

Jamaican vomiting sickness

112
Q

Inherited absence of peroxisomes in all tissues cerebrohepatorenal syndrome

A

zellweger’s syndrome

113
Q

defect in peroxysomal actibation of VLCFAs leading to its accumulation in blood and tissues

A

x-linked adrenoleukodystrophy

114
Q

where are ketone bodies utilized

A
  • brain
  • renal cortex
  • skeletal muscle
115
Q

Rate limiting step enzyme of ketone body synthesis

A

HMG CoA synthase

116
Q

substrate of ketone body synthesis

A

acetyl-CoA

117
Q

Lack of this enzyme is reason why liver cannot use ketone bodies for fuel

A

THIOPHORASE succinyl CoA acetoacetyl CoA transferase

118
Q

Nitroprusside test is a test for

A

ketones does not detect B hydroxybutyrate

119
Q

of carbons in cholesterol

A

27

120
Q

Rate limiting step enzyme of cholesterol synthesis

A

HMG CoA reductase (HMGCoA –> mevalonate)

121
Q

where does cholesterol synthesis occur

A

all cells sER

122
Q

substrates cholesterol synthesis

A

acetyl CoA NADPH ATP

123
Q

products cholesterol synthesis

A

lanosterol -> cholesterol

124
Q

competitive inhibitors of HMG CoA synthetase

A

statins

125
Q

intestinal bacteria can reduce cholesterol to

A

coprostanol cholestanol

126
Q

Rate limiting enzyme of bile acid synthesis

A

cholesterol 7 a hydroxylase

127
Q

primary bile acids conjugated with ?

A

glycine and taurine to the secondary deoxycholic acid lithocholic acid

128
Q

mother hormone steroid

A

pregnenolone

129
Q

Rate limiting enzyme of steroid hormone synthesis

A

desmolase

130
Q

Blocker of steroid hormone synthesis

A

aminogluthetimide

131
Q

where steroid hormone synthesis occurs

A

SER of adrenal cortex ovaries and testes placenta

132
Q

Overview steroid hormone synthesis

A

:) p 25 topnotch

133
Q

apoprotein in chylomicrons

A

Apo B48

134
Q

apoprotein in VLDL

A

Apo B-100

135
Q

apoprotein in in HDL

A

Apo A1

136
Q

apoprotein in the liver for uptake

A

Apo E

137
Q

lipoprotein activates LCAt

A

found in HDL!! Apo A1

138
Q

intestinal malabsorption, deficiency in apo B48 and B100, hence no chylomicron no VLDL

A

Abetalipoproteinemia

139
Q

absence of Apo A1 hence no HDL

A

Familial a lipoprotein deficiency

140
Q

most abundant phospholipid

A

phosphatidylcholine

141
Q

phospholipid plays a role in apoptosis

A

phosphatidylserine

142
Q

the only antigenic phospholipid found only in mitochondria

A

cardiolipin

143
Q

decrease in ___ causes mitochondrial dysfunction in aging and heart failure

A

cardiolipin

144
Q

sphingosine + FA

A

ceramide

145
Q

ceramide + glu or gal

A

cerebroside

146
Q

ceramide + oligosaccharide

A

globoside

147
Q

ceramide + N acetyl neuramic acid

A

ganglioside

148
Q

ceramide + sulfated galactose

A

sulfatides

149
Q

lipid storage diseases, abnormal lipid DEGRADATION in lysosomes however synthesis is normal

A

sphingolipidoses

150
Q

enzyme deficient in Tay Sach’s disease

A

Hexosaminidase A

151
Q

enzyme deficient in Fabry’s disease

A

a- Galactosidase

152
Q

enzyme deficient in Farber’s disease

A

Ceramidase

153
Q

Deficiency in B galactosidase

A

Krabbe’s disease

154
Q

Deficiency in B- glucosidase, hepatosplenomegaly erosion of long bones normal mentality

A

Gaucher’s

155
Q

deficient in Niemann Pick disease

A

sphingomyelinase

156
Q

sphingolipidoses with triad of 1. skin rash 2. hoarseness 3. bone malformatin

A

Farber’s disease

157
Q

sphingolipidoses with the 3 Rs

  • recessive x linked
  • rash
  • renal failure
A

Fabry’s disease

158
Q

All sphingolipidoses are autosomal except for ___ which is

A

fabry’s disease x linked recessive

159
Q

dietary precursor of eicosanoids

A

linoleic acid linoleic acid –> arachidonic acid –> :)

160
Q

amino acid used in the first step of heme synthesis

A

GLYCINE glycine + succinyl CoA –> d-ALA

161
Q

amino acid used in purine synthesis

A

glycine

162
Q

amino acid which carries nitrogen from peripheral tissue to liver

A

alanine

163
Q

BCAAs

A

VAL

164
Q

deficiency in branched chain a-ketoacid dehydrogenase

A

MSUD

165
Q

amino acid with longest side chain

A

Trp

166
Q

precursor of homocysteine

A

methionine

167
Q

the imino acid

A

proline

168
Q

found at phosphorylation site of enzyme modification

A

serine

169
Q

where does O linked glycosylation occur

A

Golgi apparatus

170
Q

where does N linked glycosylation occur

A

ER

171
Q

amino acid for O linked glycosylation

A

serine threonine

172
Q

amino acid for N linked glycosylation

A

asparagine

173
Q

precursor for GABA

A

glutamate

174
Q

test for folic acid deficiency ; as they excrete huge amounts of this in the urine after harge doses of Histidine

A

N-FIGlu test

175
Q

part of aminoacid that accepts protons

A

amino group

176
Q

part of amino acid that donates protons

A

carboxylic acid

177
Q

Conditionally essential amino acids

A

Arginine Histidine

178
Q

form of bonding in peptide bond

A

covalent

179
Q

configuration of all amino acids in proteins

A

L config

180
Q

reagent to remove C terminal amino acid

A

hydrazine carboxypeptidase

181
Q

reagent to remove N terminal amino acid

A

sanger’s reagent edman’s reagent phenylisothiocyanate

182
Q

amino acids per turn of spiral in alpha helix

A

3.6 aa

183
Q

combinations of adjacent secondary structures such as

B-a-B unit, greek key, B meander

A

supersecondary structures

184
Q

stabilizes the tertiary structures

A
  • disulfide bonds
  • hydrophobic reactons
  • H bonds
  • ionic bonds
185
Q

functional and three dimensional structural units of a polypeptide

A

domains

186
Q

Heme is a complex of ______ and _____

A

protoporphyrin IX ferrous iron

187
Q

what is the major transporter of carbon dioxide in the blood

A

bicarbonate 75% hemoglobin (minor)

188
Q

T (taut) form has low or high oxygen affinity?

A

low affinity

189
Q

increase in protons shifts the equilibrium to the right whereas an increase in pO2 shifts the equilibrium to the left

A

Bohr effect

190
Q

stabilizes the T structure of hemoglobin by creating more salt bridges

A

2,3 bisphosphoglycerate

191
Q

antidote methemogobinemia

A

methylene blye

192
Q

HbA1C, glucose glycosylates?

A

e-amino group of lysine

193
Q

Diagnosed by osmotic fragility test

A

Hereditary spherocytosis

194
Q

glu —> lysine substitution of Hb

A

Hb C disease

195
Q

inadequate synthesis of alpha chains of hemoglobin

A

alpha thalassemia

196
Q

Amino acids found in collagen

A

Glycine and Proline Gly-X-Y X= proline Y- hydroxyproline or hydroxylysine

197
Q

Collagen in skin and blood vessels

A

Type III

198
Q

Wound healing collagen

A

Type II to Type I

199
Q

Brittle bone syndrome

A

Osteogenesis Imperfecta

200
Q

Dietary deficiency of copper required by lysyl oxidase, impt in crosslinking in collagen

A

Menke’s syndrome

201
Q

Defect in Type VII collagen, characterized by skin breaks and blisters

A

Epidermolysis Bullosa

202
Q

FIBRILLIN scaphoid linked by DESMOSINE

A

elastin

203
Q

dolichostenomelia ectopia lentis

A

Marfan’s syndrome

204
Q

at risk for berry aneurisms

A

ehler’s danlose syndrome

Z_Biochem (8 decks)

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