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Biochem V > Final > Flashcards

Final Flashcards

1
Q

In Alzheimers, Lewy Bodies are composed of what type of protein?

A

alpha-synuclein

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2
Q

In Alzheimers, what are the plaques made of? The fibrils?

A

plaques = beta-amyloid

fibrils = Tau

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3
Q

In sickle cells anemia, what amino acid is mutated?

A

glutamate to Valine

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4
Q

What region of a protein do chaperones bind?

A

hydrophobic

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5
Q

Scurvy features a defect in what protein?

A

hydroxyproline

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6
Q

What class of molecules feature an ether linkage between two components?

A

plasmalogens

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7
Q

What receptor do alpha-neurotoxins target and destroy?

A

NAchR

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8
Q

Atrial natriuretic peptide features what type of guanylate cyclase?

A

soluble guanylate cyclase

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9
Q

What G-protein does cholera toxin target? What modification does it add to these G-protein?

A

Gs

ADP-ribosylation (turn on)

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10
Q

What G-protein does pertussis target? What modification does it add to these G-protein?

A

Gi

ADP-ribosylation (turned off)

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11
Q

What toxin does yersenia pestis produce? What does this toxin do?

A

YopH

tyrosine phosphatase

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12
Q

Pellagra features a defect in what product?

A

Niacin

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13
Q

What is B5 known as?

A

pantothenic acid

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14
Q

What is the PDH inhibited by?

A

NADH, acetyl-CoA and citrate

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15
Q

Wernicke Korsakoff features a defect in what molecule?

A

thiamine

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16
Q

What two enzymes require thiamine?

A

PDH and alpha-KG

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17
Q

What amino acid is mitated in MERRF?

A

lysine tRNA

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18
Q

Crigler-Najjar Syndrome (Gilbert Disease) features a defect in what protein?

A

glucoronosyltransferase

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19
Q

FA degradation disorders present with what type of observation?

A

hypoglycemia and low ketone bodies

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20
Q

FA degradation requires what kind of bond?

A

trans

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21
Q

alpha-oxidation degrades what kind of fatty acids?

A

branched chain

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22
Q

acute fatty liver of pregnancy cant degrade what molecule?

A

long chain fatty acids

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23
Q

Refsum disease features defects in degrading what kind of molecule?

A

branched chain FAs

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24
Q

In the ER, what molecule is used as a donor for FA synthesis? Mitochondria?

A

malonyl-CoA

acetyl-CoA

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25
Q

What is the committed step in ketone body synthesis?

A

HMG CoA Lyase

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26
Q

Where does FA desaturation occur?

A

ER

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27
Q

What is the reguated step of FA degradation?

A

CPT-1

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28
Q

PPAR activates genes required for what?

A

fatty acid oxidation and ketone

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29
Q

What is the committed step of FA synthesis?

A

acetyl-CoA Carboxylase

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30
Q

What protein kinase activates HSL?

A

PKA

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31
Q

What is the function of DGAT?

A

to transfer a fatty acid to DAG

committed step of FA acid synthesis

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32
Q

Which side, cytosolic or luminal, are negatively charged compounds found?

A

cytosolic face

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33
Q

What are leukotrienes synthesized from?

A

arachidonic acid

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34
Q

What composes lactoce?

A

glucose and galactose

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35
Q

What tissues participate in the glycerol phosphate shuttle?

A

muscle and brain

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36
Q

WHat tissues participate in the malate aspartate shuttle?

A

liver and heart

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37
Q

What is the most regulated enzyme of gluconeogenesis?

A

F16bPase

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38
Q

Arseate bypasses what enzyme of glycolysis?

A

GAPDH

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39
Q

What co-factor is required for the non-oxidative phase of the PPP?

A

thiamine

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40
Q

What enzyme converts G6P to G1P?

A

phosphoglucomutase

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41
Q

What two receptors/enzymes of GNG do muscles lack?

A

glucagon receptor and G6Pase

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42
Q

Van Gierke defect?

A

G6Pase

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43
Q

Cori Disease defect?

A

debranching enzyme

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44
Q

Pompe disease defect?

A

1,4-glucosidase

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45
Q

McArdle disease defect?

A

glycogen phosphorylase

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46
Q

Hunter syndrome defect?

A

Iduronate Sulfatase

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47
Q

Hurler syndrome defect?

A

a-Iduronase

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48
Q

San Filippo defect?

A

Heperan sulfate

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49
Q

What sugars are attached to ceramide to form cerebroside?

A

glucose or galactose

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50
Q

What sugars are attached to ceramide to form globosides?

A

2-4 neutral sugars

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51
Q

What sugars are attached to ceramide to form gangliosides?

A

NANA

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52
Q

What enzyme is deficient in Gaucher’s Disease?

A

beta-glucosidase

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53
Q

What is the committed step of cholesterol synthesis?

A

HMG CoA Reductase

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54
Q

What is the first product committed to cholesterol synthesis?

A

Mevalonic Acid (Mevalonate)

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55
Q

What cofactor do all the aminotransferases have a requirement for?

A

pyridoxal phosphate (B6)

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56
Q

Serine Hydroxymethyltransferase catalyzes what reaction? What is produced?

A

Ser –> Gly

THF

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57
Q

What enzyme is deficient in homocystinuria?

A

cystathione beta synthase

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58
Q

What enzyme is defective in familial hyperinsulinemic hypoglycemia? What is the defect?

A

glutamate dehydrogenase

glutamate is not inhibited by GTP

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59
Q

What cofactor does the glycine cleavage pathway produce?

A

N510 THF

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60
Q

What enzyme metabolizes Propionyl-CoA?

A

propionyl CoA Carboxylase

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61
Q

What is propionyl CoA eventually converted into?

A

succinyl CoA

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62
Q

What co-factor is required to convert Propionyl-CoA into Succinyl-CoA?

A

B12

63
Q

What is cofactor does methionine synthase produce?

A

THF

64
Q

What does methionine synthase convert into methionne?

A

homocysteine

65
Q

What enzyme is defective in tyrosinemia II?

A

tyrosine aminotransferase

66
Q

What enzyme is deficient in alkaptonuria?

A

homogentisate oxidase

67
Q

What enzyme is deficient in tyrosinemia I?

A

fumarylacetoacetate hydrolase

68
Q

What enzyme catalyzes the committed step of pyrimidine synthesis?

A

amidophosphoribosyltransferase

69
Q

What enzyme is deficient in orototic aciduria?

A

UMP Synthase (OMP Decarboxylase and Orotate Phosphoribosyl Transferase)

70
Q

What is broken down into beta-aminoisobutyrate?

A

dTMP

71
Q

What is broken down into beta-alanine?

A

all other pyrimidines

72
Q

HGPRTase salvages what purines?

A

hypoxanthine and guanine

73
Q

What enzymes salvages adenine?

A

APRTase

74
Q

What vitamin contains a series of unconjugated double bonds?

A

Vitamin A

75
Q

What lipid breakdown product is indicative of oxidative stress?

A

malondialdehdye

76
Q

What protein controls G1/S?

A

RB-E2F

77
Q

What proteins controls S?

A

P27-KIP1

78
Q

What protein controls G2/S?

A

Cdc25c

79
Q

What does Cdc25c activate?

A

1-A/B

80
Q

What does Cdc25a activate?

A

2-A/E

81
Q

What activates p53? What does p53 activate?

A

ATM/ATR

p21-CIP1

82
Q

What does beta-catenin active?

A

Myc and Cyclin D

83
Q

What oncogene is translocated in Burkitts Lymphoma?

A

c-Myc from chromosome 8 to chromosome 14

84
Q

What does Mdm2 serve to do in regards to p53?

A

E3 ubiquitin ligase

85
Q

What does NF1 function to do?

A

increase GTP hydrolysis of Ras

86
Q

What chromosomal aberration is present in chronic myeloid leukemia?

A

philadelphia chromosome

87
Q

What DNA polymerase contaisn exonuclease activity?

A

DNA polymerase delta

88
Q

Defects in what process are inhibited in xeroderma pigmentosum?

A

nucleotide excision repair

89
Q

What is cytarabine an analogue of?

A

cytosine

90
Q

WHat is cytarabine used to treat?

A

acute leukemia

91
Q

What is cyclophosphamide used to treat? What is the mechanism of action of cyclophosphamide?

A

Hodgkins lymphoma

DNA intercalating agent

92
Q

What TFII contains helicase activity?

A

TFIIH

93
Q

What does alpha-amanitin inhibit?

A

eukaryotic RNA Pol II

94
Q

What signals SREBP1a to translocate to the nucleus?

A

low intracellular cholesterol levels

95
Q

What is the mechanism of action of tetracycline?

A

inhibits tRNA from entering the A site

96
Q

E2F can be inhibited by what toxin?

A

diptheria

97
Q

Myristoylation occurs on what residues?

A

N-terminal glycines

98
Q

Where are isoprenoids attached?

A

cyteines at C terminus

99
Q

Where are palmitic acids attached?

A

cysteines throughout the protein

100
Q

What residues signal long life of proteins? What residues singla short life of protein?

A

Ser or Met

short = Lys or Arg

101
Q

Gain of function RET = ?

A

multiple endocrine neoplasia

102
Q

Loss of function RET = ?

A

Hirchsprung Disease

103
Q

LHON affects what mitochondrial complex?

A

complex I

104
Q

what proteins bring about de novo methylation of protein?

A

DNMT3a and DNMT3b

105
Q

Beckwith-Wiedemann = ?

What protein is over-expressed in Beckwith-Wiedemann?

A

two copies of paternal 11

IGFR2

106
Q

HDAC inhibitors treat?

A

chronic lymphocytic leukemia

107
Q

DNMT inhibitor = ?

What does it treat?

A

5-AZT

acute myeloid leukemia

108
Q

What do noggin/chordin do?

A

induce dorsal development

109
Q

What is the function of HOX?

A

patterning along the anterior and posterior axis

110
Q

What defects are seen with aberrant Shh signaling?

A

midface and forebrain

111
Q

What is the function of Nodal?

A

left looping of the heart

112
Q

What is the fuction of HOX11/13?

A

distal development of limbs

113
Q

Smith Lemli Opitz syndrome affects the signaling of what molecule?

A

Shh

114
Q

Hematochromatosis protects against?

A

Plague

115
Q

CFTR protects against?

A

typhoid

116
Q

Where is HMG-CoA Lyase expressed?

A

liver and kidney

117
Q

What does Phenylalanine Hydroxylase oxidize?

A

Tetrahydrobiopterin

118
Q

What kind of FAs get funneled to propionyl-CoA?

A

odd chain fatty acids

119
Q

What is the early response gene? What does the early response gene transcribe?

A

c-Fos

Mid and Late G1 cyclins

120
Q

What do CBP and EP300 function as?

A

HAT

121
Q

Bcr-Abl fusion activates what?

A

soluble protein tyrosine kinase

122
Q

What is Rituximab used to treat?

A

non-Hodgkins Lymphoma

123
Q

What drug is used to treat Hodgkins Lymphoma?

A

cyclophosphamide

124
Q

What enzyme converts hypoxanthine to xanthine?

A

xanthine oxidase

125
Q

What enzyme converts xanthine to uric acid?

A

xanthine oxidase

126
Q

What reaction does amidophosphoribosyltranse catalyze?

A

PRPP to PRA

127
Q

What is the committed step of heme synthesis?

A

ALA synthase

128
Q

What does ribonucleotide reductase oxidize?

A

thioredoxin

129
Q

What enzyme does hydroxyurea inhibit?

A

ribonucleotide reductase

130
Q

What is the first enzyme involved in gluconeogenesis?

A

pyruvate carboxylase

131
Q

Sphingolipids are synthesized from what two molecules?

A

Palmitoyl-CoA and Serine

132
Q

What does E2F active?

A

CDK2-A/E

133
Q

What inhibits p27-KIP1?

A

E/CDK2

134
Q

What does p27KIP-1 Inhibit?

A

A/CDK2

135
Q

What enzyme catalyzes R5P to PRPP?

A

PRPP synthetase

136
Q

What inhibits the PDH?

A

NADH, citrate and acetyl-CoA

137
Q

Is the PDH active with it is phosphorylated or de-phosphorylated?

A

de-phosphorylated

138
Q

What reaction does phospho-mannoseisomerase catalyze?

A

F6P to M6P

139
Q

What amino acid does arginosuccinate synthetase require?

A

Aspartate

140
Q

What is the early response gene?

A

c-Fos

141
Q

What enzyme is responsible for the salvage of pyrimidines? What pyrimidine is not salvaged?

A

pyrimidine phosphoribosyltransferase

cytosine

142
Q

What enzyme of the PDH contains thiamine?

A

pyruvate decarboxylase

143
Q

What catalyzes the reaction of UTP to CTP?

A

CTP synthase

144
Q

What is the product of HMG-CoA Lyase?

A

Aceto-acetate

145
Q

What is the product of Acetyl-CoA Thiolase?

A

aceto-acetyl-CoA

146
Q

What is the final enzyme in ketone body synthesis?

A

beta-hydroxy-butyrate dehydrogenase

147
Q

Regarding the G2/M checkpoint, what protein can inhibit the action of Cdc25c?

A

ATM

148
Q

What can ATM detect?

A

double-stranded breaks

149
Q

What activates BAX?

A

p53

150
Q

What does BAX activate?

A

cyt C

151
Q

What does cyt C activate?

A

APAF-1

152
Q

What does APAF-1 activate?

A

caspase 9

153
Q

What is the early response gene? What two genes contribute to the early response gene?

A

Fos

Jun and Myc

Biochem V (11 decks)

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