Topnotch 2 Flashcards

1
Q

Protein turnover per day

A

200-300/day

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2
Q

Protein degradation mechanism inside the cell, using non energy dependent degradative enzyme

A

ubiquitin proteosome mechanism

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3
Q

Manner of excretion of excess nitrogen by humans

A

Ureotelic

using urea

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4
Q

2 steps in Nitrogen removal

A
  1. Transamination

2. Oxidative Deamination

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5
Q

All amino acids must transfer amino groups to a-ketoglutarate except for (2)

A

Lysine

Threonine

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6
Q

Coenzyme of ALT and AST

A
Vit B6
(Pyridoxine)
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7
Q

Asp acted by AST –>____

A

oxaloacetate

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8
Q

Ala acted upon by ALT —> _____

A

pyruvate

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9
Q

where oxidative deamination occurs

A

LIVER and KIDNEY only

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10
Q

Removal of excess N from peripheral tissues is through

A

GLUTAMINE

garbage collector

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11
Q

where urea cycle occurs

A

liver

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12
Q

Donors of atoms of Urea

A

NH3 from ammonia
NH3 from aspartate
CO2

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13
Q

Enzyme in rate limiting step of Urea cycle

A

CPS-I

Carbamoyl Phosphate Synthase I

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14
Q

Reactions in Urea Cycle

A

Ordinary Careless Crappers Are Also Frivolous About Urination

Ornithine +
Carbamoyl Phosphate= Citruline

Citruline
+ Aspartate –>Arginosuccinate

Fumarate + Arginine

Arginine = Urea + Ornithine

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15
Q

Energy requirement of urea cycle

A

4 ATPs

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16
Q

Enzyme defect in Hereditary Hyperammonemia Type I (most common), presenting with lethargy, vomiting, coma and death

A

CPS I

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17
Q

Enzyme defect in Hereditary Hyperammonemia Type II

A

Ornithine transcarbamoylase

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18
Q

Purely ketogenic amino acids

A

Leucine

Lysine

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19
Q

Amino acids both ketogenic and glucogenic

A

Phenylalanine
Tyrosine
Tryptophan
Isoleucine

FYI, W

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20
Q

Yield by ketogenic amino acid metabolism

A

acetoacetate

acetyl CoA

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21
Q

Entry point in Krebs is Fumarate

A

Phenylalanine and Tyrosine

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22
Q

Entry point in Krebs of

Glutamine, Glutamate, Proline, Arginine, Histidine

A

a ketoglutarate

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23
Q

Entry point in Krebs of Methionine, Valine. Isoleucine, Threonine

A

Succinyl CoA

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24
Q

Entry point in Krebs is oxaloacetate

A

Aspartate, Asparagine

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25
Q

Amino acid Glycine is made from

A

Serine

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26
Q

Proline is synthesized from

A

Glutamine

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27
Q

Serine is synthesized from

A

3-phosphoglycerate

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28
Q

Cofactor of Phe hydroxylase hence deficiency can manifest like PKU

A

Tetrahydrobipterin

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29
Q

Alkaptonuria is due to congenital deficiency of _____

A

Homogentisic Acid Oxidase

in the degradative pathway of Tyrosine

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30
Q

darkening of connective tissue seen in alkaptonuria

A

ochronosis

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31
Q

congenital deficiency of TYROSINASE or defective TYROSINE transporters

A

Albinism

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32
Q

Occurs to either:
cystathionine synthase deficiency
or its dec affinity for Vit B6
homocysteine methyltransferase deficiency

A

homocystinuria

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33
Q

Causes downward and inward lens subluxation, tall stature, MR

A

Homocytinuria

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34
Q

Defect of tubular amino acid transporter for COLA

A

cystinuria

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35
Q

Treatment of Cystinuria

A

Acetazolamine (to alkalinize the urine)

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36
Q

Enzyme deficient in MSUD

A

a-ketoacid dehydrogenase

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37
Q

Predominant sites of heme synthesis

A

Liver

Bone marrow

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38
Q

Rate limiting step of Heme Synthesis

A

Formation of delta aminolevulinic acid

catalyzed by ALA synthase

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39
Q

Co-factor of ALA synthase (*heme synthesis)

A

pyridoxine

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40
Q

Photosensitivity
Abdominal pain
Neuropsych symptoms
Most common porphyria

A

porphyria cutanea tarda

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41
Q

reaction heme synthesis

A

glycine + succinyl CoA —> d-aminolevulinic acid

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42
Q

Enzyme of heme synthesis inhibited by zinc

A

ALA dehydratase

in the formation of porphobilinogen

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43
Q

Heme synthesis, start of the RING

A

Uroporphyrinogen

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44
Q

Enzyme of heme synthesis inhibited by lead

A

ferrochelatase,

ALA dehydratase

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45
Q

How is heme formed

A

(Fe 3+) into protoporphyrin IX

by heme synthase

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46
Q

Blood picture pyridoxine deficiency associated with isoniazid therapy

A

Sideroblastic anemia with ringed sideroblasts

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47
Q

Wrist drop, claw hand

basophilic stippling RBC

A

lead poisoning

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48
Q

start of photosensitivity enzyme heme synthesis

A

Uroporphyrinogen decarboxylase

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49
Q

Glucoronic acid + N acetyl glucosamine

A

Hyaluronic acid

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50
Q

GAG Vitreous humor, synovial fluid

A

Hyaluronic acid

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51
Q

GAG Glucoronic acid + N acetyl galactosamine

A

Chondroitin Sulfate

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52
Q

GAG Iduronate + Glucosamine

A

Heparin

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53
Q

All GAGs covalently attached to protein to form proteoglycans except

A

Heparin

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54
Q

GAG in endochondral bone also cartilage

A

chondroitin sulfate

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55
Q

GAGs role in corneal transparency

A

Keratan sulfate I

Dermatan sulfate

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56
Q

GAG impt in charge of glomerulus

A

Heparan sulfate

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57
Q

GAG cell migration in wound repair

compressibility of cartilage

A

Hyaluronic acid

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58
Q

Where GAGs are synthesized

A

ER

by acid hydrolases

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59
Q

Accumulation of GAGs in lysosomes due to deficiency in hydrolases

A

Mucopolysaccharidoses

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60
Q

Mucopolysaccharidoses all are autosomal recessive except

A

Hunters

X linked recessive

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61
Q

Mucopolysaccharidoses all have MENTAL RETARDATION except

A

Morquio’s syndrome

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62
Q

Deficient in Morquio’s syndrome

A

galactose-6-sulfatase

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63
Q

Deficient in Hurler’s syndrome

A

a-L-iduronidase

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64
Q

Type II Mucopolysaccharidoses

A

Sanflippo syndrome

so many!! refer to p 7 handout 2

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65
Q

Function of glycoproteins

A

cell recognition

cell antigenicity

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66
Q

Deficient phosphorylation of mannose residues in N linked glycoprotein pre enzymes

A

I-cell disease

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67
Q

protein HIV

A

gp120 + p41

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68
Q

Malaria glycoprotein

A

GPI

69
Q

Type A glycosphingolipids

A

N acetyl galactosamine

70
Q

Type B glycosphingolipids

A

Galactose

71
Q

of AAs in insulin

A

51

72
Q

where the 3 disulfide bonds in insulin is found

A

A7 -B7 - interchain
A20- B19- interchain
A6-A11- intrachain

73
Q

sperm receptor

A

zP3

74
Q

Glucagon receptor

A

Gs

inc cAMP

75
Q

nutrients requirement 50% of healthy individuals

A

Estimated Average Requirement

76
Q

nutrients requirement 95% of healthy individuals

A

Rec Daily Allowance

77
Q

Estimated energy requirement

Sedentary/ Moderately Active/Very Active

A

30/ 35/ 40

78
Q

Basal Metabolic Rate

A

60%

79
Q

Form of Vit A growth regulators in epithelium

A

retinoic acid

80
Q

Form of Vit A gametogenesis in gonads

A

retinol

81
Q

Form of Vit A rod and cones cells for vision

A

retinal

82
Q

Toxicity of Vit A

A

Hyperkeratosis
Hepatomegaly
Pseudotumor cerebri

83
Q

active form Vit D

A

Calcitriol (1,25 (OH) Vit D2)

84
Q

Vit D3 hydroxylated 2x how

A

25 hydroxylation in liver

1 hydroxylation in kidney

85
Q

Long QT. Calcium is?

A

low

86
Q

Most toxic vitamin

A

Vitamin D

87
Q

Synthetic Vit K

A

Manadione

88
Q

Vit K given to newborns

A

K1 Phylloquinone

89
Q

Vitamin transketolase reactions in HMP

A

Thiamine

90
Q

Vitamin def
confusion
loss of muscle coordination

A

Wernickes

91
Q

Vitamin def
inability to form new memories
confabulation

A

Korsakoff

92
Q

Vitamin in FAD

A

Riboflavin (Vit B2)

93
Q

Vit def stomatitis

cheilosis

A

Vit B2

94
Q

Vitamin can be derived from Trp

A

Niacin

95
Q

Trp in carcinoid syndrome

A

increase Trp metabolism

96
Q

Trp in Hartnup Disease

A

Dec Trp absorption

97
Q

Vitamin used as cofactor for acyl transfers

A

Pantothenic acid

98
Q

Vitamin no deficiency state

A

Vit B2, B5

99
Q

Vitamin used for methionine synthesis

A

Vit B12

100
Q

Vitamin cofactor by dopamine hydroxylase

A

Vit C

101
Q

Deficiency of this Vitamin is induced by AVIDIN in egg whites

A

Biotin

102
Q

Inhibits dihydropteroate synthase

A

sulfonamides (sulfamethoxazole)

103
Q

Inhibits dihydrofolate reductase

A

trimethoprim

104
Q

Isoniazid is activated by Phase I or Phase II reaction?

A

Phase II (Acetylation)

105
Q

Link in NucleoSIDE between sugar and purine/pyrimidine

A

B-N-glycosidic bond

106
Q

Nucleoside with phosphoryl group at C5

A

nucelotides

107
Q

Purine synthesis

Source of atoms

A
Glutamine
Aspartic Acid
Carbon Dioxide
Glycine
THF
(GAC + GT)
108
Q

Pyrimidine synthesis

Source of atoms

A

Glutamine
Aspartic Acid
Carbon Dioxide
(GAC)

109
Q

Committed step in De Novo Purine Synthesis

A

PRPP —> 5-phosphoribosylamine

catalyzed by glutamyl PRPP amidotransferase

110
Q

Parent purine nucleotide catalyzed in NINE steps

A

IMP

111
Q

source of ribose 5 phosphate in purine salvage pathway

A

PRPP

112
Q

Salvage pathway enzyme adenine to AMP

A

adenine phosphoribosyltransferase (APRT)

113
Q

Salvage pathway enzyme guanine to GMP

A

Hypoxanthine Guanine phosphoribosyltransferase

114
Q

Salvage pathway enzyme hypoxanthine to IMP

A

Hypoxanthine Guanine phosphoribosyltransferase (HGPRT)

115
Q

Rate limiting step of De Novo Pyrimidine Synthesis

A

CPS II

116
Q

Which of the CPS is found in cytoplasm?

mitochondria?

A

mitochondria CPS I (urea cycle)

cytoplasm CPS II (pyr synthesis)

117
Q

Inhibits CPS II

A

UTP

118
Q

Hyperuricemia
MR
Self Mutilation

A

Lesch Nyhan

119
Q

parent pyrimidine

A

OMP

orotidine monophosphate

120
Q

Steps pyrimidine de novo synthesis

A

:)
OMP –OMP decarboxylase–> UMP
CTP synthase: UTP and CTP
dUMP -thymidylate synthase - dTMP

121
Q

Thymineless death

A

5-FU

122
Q

enzyme for reduction of nucleosides to DEOXY forms

A

ribonucleotide reductase

123
Q

Enzyme which regenerates the reduced forms of ribonucleotide reductase

A

thioredoxin

124
Q

reconverts thioredoxin to reduced form

A

NADPH

125
Q

action xanthine oxidase

A

xanthine to uric acid

126
Q

Products of pyrimidine degradation

A

B-alanine

B-aminoisobutyrate

127
Q

PABA analogs

A

sulfonamides

128
Q

explain the purine overproduction in Von Gierke

A

deficiency in glucose-6-phosphatase, hence glu-6-P is shunted to generation of PRPP precursor r-5-P

129
Q

Pathophysiology of ADA deficiency

A

ADA is for Adenosine to inosine

deficiency: very high dATP which inhibits ribonucleotide reductase

130
Q

abnormal growth
megaloblastic anemia
excrete orotate

A

orotic aciduria

131
Q

Right handed helix with 10 residues per 360 deg

A

B DNA

132
Q

Right handed with 11 base pairs per turn

A

A DNA

133
Q

left handed helix 12 base pairs per turn

A

z DNA

134
Q

Amino acids high in nucleosome

A

Arginine

Lysine

135
Q

densely packed

transcriptionally inactive chromatin

A

heterochromatin

136
Q

transcriptionally active chromatin

stains less densely

A

euchromatin

137
Q

Blot for DNA

A

southern blot
RNA- Northern
Protein- Western

138
Q

Dx tool for single base changes

deletion/ insertion

A

Restriction Fragment Length Polymorphism

139
Q

Enzyme DNA leading strand synthesis prokaryotes and eukaryotes

A

DNA pol III iin 3’ to 5’

140
Q

Prokaryotic DNA synthesis removes the RNA primers through its 5’-3’ exonuclease activity, gap filling

A

DNA pol I

141
Q

Prokaryotic DNA synthesis Nicks between okazaki fragments

A

DNA ligase

142
Q

Prokaryotic DNA synthesis

Recognizes the origin of replication

A

DNA A protein

143
Q

Prokaryotic DNA synthesis

maintains separation of parental strand after action of DNA helicase ( which unwinds DNA)

A

single stranded DNA binding proteins

144
Q

Prokaryotic DNA synthesis

removes supercoils

A

DNA topoisomerase

145
Q

DNA proof reading and repair

A

DNA pol II

146
Q

highly repetitive DNA found at ends of chromosomes

A

telomeres

147
Q

mismatch repair

A

HNPCC

148
Q

Thymine dimers defect

A

xeroderma pigmentosum

149
Q

post transcriptional modifications

A

poly A tail
methylguanosine cap
splicing

150
Q

post transcriptional modifications

A

poly A tail
methylguanosine cap
splicing

151
Q

In transcription: where the RNA polymerase holoenzyme binds

A

Pribnow box (prokaryotes) 8-10 nucleotides left
-35 sequence 35 bases left
TATA or Hogness box, CAAT, GC box (eukaryotes)

152
Q

In transcription: how is it terminated

A
  1. by RNA pol alone (palindrome, hairpin loop)

2. rho factor binds at newly syn RNA

153
Q

intervening sequence in synthesized DNA

A

intron

154
Q

RNA polymerases in eukaryotes (as there are 3, unlike in prokaryotes)

A

RNA pol I- large rRNAs
RNA pol II- mRNAs
RNA pol III- tRNAs

155
Q

RNA polymerases in eukaryotes (as there are 3, unlike in prokaryotes)

A

RNA pol I- large rRNAs
RNA pol II- mRNAs
RNA pol III- tRNAs

156
Q

structural genes coding for group of proteins required for a particular function

A

operon

157
Q

of Codons

A

64 codons

158
Q

Translation: Stop codons

A

UAA
UGA
UAG

159
Q

Translation: how is it initiated?

A

after charging,
initiation factors eIFs assemble the 40s ribosome and initiator tRNA (methionine)
eukaryotes 60s + 40s –> 80s
prokaryotes 50s + 30s –> 70s

160
Q

Translation: how is it initiated?

A

after charging,
initiation factors eIFs assemble the 40s ribosome and initiator tRNA
eukaryotes 60s + 40s –> 80s
prokaryotes 50s + 30s –> 70s

161
Q

Translation: catalyzes peptide bond formation

A

peptidyltransferase

162
Q

Translation: how is it terminated?

A

when a stop codon binds to the A site

163
Q

proteins defective/ meant for degradation is bound to

A

ubiquitin

164
Q

Antibiotic preventing binding of tRNAs to A site

A

Tetracycline

165
Q

Antibiotic preventing binding of tRNAs to A site

A

Tetracycline

166
Q

Antibiotic inhibiting peptide bond formation (transpeptidase)

A

chloramphenicol

167
Q

Antibiotic binds to 50s inhibiting translocation

A

Clindamycin

Erythromycin

168
Q

Antibiotic

Binds to DNA dep RNA pol thus inhibiting RNA synthesis

A

Rifampicin