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Flashcards in Final Deck (153):
1

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

alpha-synuclein

2

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

plaques = beta-amyloid

fibrils = Tau

3

In sickle cells anemia, what amino acid is mutated?

glutamate to Valine

4

What region of a protein do chaperones bind?

hydrophobic

5

Scurvy features a defect in what protein?

hydroxyproline

6

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

plasmalogens

7

What receptor do alpha-neurotoxins target and destroy?

NAchR

8

Atrial natriuretic peptide features what type of guanylate cyclase?

soluble guanylate cyclase

9

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

Gs

ADP-ribosylation (turn on)

10

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

Gi

ADP-ribosylation (turned off)

11

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

YopH

tyrosine phosphatase

12

Pellagra features a defect in what product?

Niacin

13

What is B5 known as?

pantothenic acid

14

What is the PDH inhibited by?

NADH, acetyl-CoA and citrate

15

Wernicke Korsakoff features a defect in what molecule?

thiamine

16

What two enzymes require thiamine?

PDH and alpha-KG

17

What amino acid is mitated in MERRF?

lysine tRNA

18

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

glucoronosyltransferase

19

FA degradation disorders present with what type of observation?

hypoglycemia and low ketone bodies

20

FA degradation requires what kind of bond?

trans

21

alpha-oxidation degrades what kind of fatty acids?

branched chain

22

acute fatty liver of pregnancy cant degrade what molecule?

long chain fatty acids

23

Refsum disease features defects in degrading what kind of molecule?

branched chain FAs

24

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

malonyl-CoA

acetyl-CoA

25

What is the committed step in ketone body synthesis?

HMG CoA Lyase

26

Where does FA desaturation occur?

ER

27

What is the reguated step of FA degradation?

CPT-1

28

PPAR activates genes required for what?

fatty acid oxidation and ketone

29

What is the committed step of FA synthesis?

acetyl-CoA Carboxylase

30

What protein kinase activates HSL?

PKA

31

What is the function of DGAT?

to transfer a fatty acid to DAG

committed step of FA acid synthesis

32

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

cytosolic face

33

What are leukotrienes synthesized from?

arachidonic acid

34

What composes lactoce?

glucose and galactose

35

What tissues participate in the glycerol phosphate shuttle?

muscle and brain

36

WHat tissues participate in the malate aspartate shuttle?

liver and heart

37

What is the most regulated enzyme of gluconeogenesis?

F16bPase

38

Arseate bypasses what enzyme of glycolysis?

GAPDH

39

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

thiamine

40

What enzyme converts G6P to G1P?

phosphoglucomutase

41

What two receptors/enzymes of GNG do muscles lack?

glucagon receptor and G6Pase

42

Van Gierke defect?

G6Pase

43

Cori Disease defect?

debranching enzyme

44

Pompe disease defect?

1,4-glucosidase

45

McArdle disease defect?

glycogen phosphorylase

46

Hunter syndrome defect?

Iduronate Sulfatase

47

Hurler syndrome defect?

a-Iduronase

48

San Filippo defect?

Heperan sulfate

49

What sugars are attached to ceramide to form cerebroside?

glucose or galactose

50

What sugars are attached to ceramide to form globosides?

2-4 neutral sugars

51

What sugars are attached to ceramide to form gangliosides?

NANA

52

What enzyme is deficient in Gaucher's Disease?

beta-glucosidase

53

What is the committed step of cholesterol synthesis?

HMG CoA Reductase

54

What is the first product committed to cholesterol synthesis?

Mevalonic Acid (Mevalonate)

55

What cofactor do all the aminotransferases have a requirement for?

pyridoxal phosphate (B6)

56

Serine Hydroxymethyltransferase catalyzes what reaction? What is produced?

Ser --> Gly

THF

57

What enzyme is deficient in homocystinuria?

cystathione beta synthase

58

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

glutamate dehydrogenase

glutamate is not inhibited by GTP

59

What cofactor does the glycine cleavage pathway produce?

N510 THF

60

What enzyme metabolizes Propionyl-CoA?

propionyl CoA Carboxylase

61

What is propionyl CoA eventually converted into?

succinyl CoA

62

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

B12

63

What is cofactor does methionine synthase produce?

THF

64

What does methionine synthase convert into methionne?

homocysteine

65

What enzyme is defective in tyrosinemia II?

tyrosine aminotransferase

66

What enzyme is deficient in alkaptonuria?

homogentisate oxidase

67

What enzyme is deficient in tyrosinemia I?

fumarylacetoacetate hydrolase

68

What enzyme catalyzes the committed step of pyrimidine synthesis?

amidophosphoribosyltransferase

69

What enzyme is deficient in orototic aciduria?

UMP Synthase (OMP Decarboxylase and Orotate Phosphoribosyl Transferase)

70

What is broken down into beta-aminoisobutyrate?

dTMP

71

What is broken down into beta-alanine?

all other pyrimidines

72

HGPRTase salvages what purines?

hypoxanthine and guanine

73

What enzymes salvages adenine?

APRTase

74

What vitamin contains a series of unconjugated double bonds?

Vitamin A

75

What lipid breakdown product is indicative of oxidative stress?

malondialdehdye

76

What protein controls G1/S?

RB-E2F

77

What proteins controls S?

P27-KIP1

78

What protein controls G2/S?

Cdc25c

79

What does Cdc25c activate?

1-A/B

80

What does Cdc25a activate?

2-A/E

81

What activates p53? What does p53 activate?

ATM/ATR

p21-CIP1

82

What does beta-catenin active?

Myc and Cyclin D

83

What oncogene is translocated in Burkitts Lymphoma?

c-Myc from chromosome 8 to chromosome 14

84

What does Mdm2 serve to do in regards to p53?

E3 ubiquitin ligase

85

What does NF1 function to do?

increase GTP hydrolysis of Ras

86

What chromosomal aberration is present in chronic myeloid leukemia?

philadelphia chromosome

87

What DNA polymerase contaisn exonuclease activity?

DNA polymerase delta

88

Defects in what process are inhibited in xeroderma pigmentosum?

nucleotide excision repair

89

What is cytarabine an analogue of?

cytosine

90

WHat is cytarabine used to treat?

acute leukemia

91

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

Hodgkins lymphoma

DNA intercalating agent

92

What TFII contains helicase activity?

TFIIH

93

What does alpha-amanitin inhibit?

eukaryotic RNA Pol II

94

What signals SREBP1a to translocate to the nucleus?

low intracellular cholesterol levels

95

What is the mechanism of action of tetracycline?

inhibits tRNA from entering the A site

96

E2F can be inhibited by what toxin?

diptheria

97

Myristoylation occurs on what residues?

N-terminal glycines

98

Where are isoprenoids attached?

cyteines at C terminus

99

Where are palmitic acids attached?

cysteines throughout the protein

100

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

Ser or Met

short = Lys or Arg

101

Gain of function RET = ?

multiple endocrine neoplasia

102

Loss of function RET = ?

Hirchsprung Disease

103

LHON affects what mitochondrial complex?

complex I

104

what proteins bring about de novo methylation of protein?

DNMT3a and DNMT3b

105

Beckwith-Wiedemann = ?

What protein is over-expressed in Beckwith-Wiedemann?

two copies of paternal 11

IGFR2

106

HDAC inhibitors treat?

chronic lymphocytic leukemia

107

DNMT inhibitor = ?

What does it treat?

5-AZT

acute myeloid leukemia

108

What do noggin/chordin do?

induce dorsal development

109

What is the function of HOX?

patterning along the anterior and posterior axis

110

What defects are seen with aberrant Shh signaling?

midface and forebrain

111

What is the function of Nodal?

left looping of the heart

112

What is the fuction of HOX11/13?

distal development of limbs

113

Smith Lemli Opitz syndrome affects the signaling of what molecule?

Shh

114

Hematochromatosis protects against?

Plague

115

CFTR protects against?

typhoid

116

Where is HMG-CoA Lyase expressed?

liver and kidney

117

What does Phenylalanine Hydroxylase oxidize?

Tetrahydrobiopterin

118

What kind of FAs get funneled to propionyl-CoA?

odd chain fatty acids

119

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

c-Fos

Mid and Late G1 cyclins

120

What do CBP and EP300 function as?

HAT

121

Bcr-Abl fusion activates what?

soluble protein tyrosine kinase

122

What is Rituximab used to treat?

non-Hodgkins Lymphoma

123

What drug is used to treat Hodgkins Lymphoma?

cyclophosphamide

124

What enzyme converts hypoxanthine to xanthine?

xanthine oxidase

125

What enzyme converts xanthine to uric acid?

xanthine oxidase

126

What reaction does amidophosphoribosyltranse catalyze?

PRPP to PRA

127

What is the committed step of heme synthesis?

ALA synthase

128

What does ribonucleotide reductase oxidize?

thioredoxin

129

What enzyme does hydroxyurea inhibit?

ribonucleotide reductase

130

What is the first enzyme involved in gluconeogenesis?

pyruvate carboxylase

131

Sphingolipids are synthesized from what two molecules?

Palmitoyl-CoA and Serine

132

What does E2F active?

CDK2-A/E

133

What inhibits p27-KIP1?

E/CDK2

134

What does p27KIP-1 Inhibit?

A/CDK2

135

What enzyme catalyzes R5P to PRPP?

PRPP synthetase

136

What inhibits the PDH?

NADH, citrate and acetyl-CoA

137

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

de-phosphorylated

138

What reaction does phospho-mannoseisomerase catalyze?

F6P to M6P

139

What amino acid does arginosuccinate synthetase require?

Aspartate

140

What is the early response gene?

c-Fos

141

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

pyrimidine phosphoribosyltransferase

cytosine

142

What enzyme of the PDH contains thiamine?

pyruvate decarboxylase

143

What catalyzes the reaction of UTP to CTP?

CTP synthase

144

What is the product of HMG-CoA Lyase?

Aceto-acetate

145

What is the product of Acetyl-CoA Thiolase?

aceto-acetyl-CoA

146

What is the final enzyme in ketone body synthesis?

beta-hydroxy-butyrate dehydrogenase

147

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

ATM

148

What can ATM detect?

double-stranded breaks

149

What activates BAX?

p53

150

What does BAX activate?

cyt C

151

What does cyt C activate?

APAF-1

152

What does APAF-1 activate?

caspase 9

153

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

Fos

Jun and Myc