Protein- Metabolism Flashcards

1
Q

Essential or Nonessential - Phenylalanine

A

Essential

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Tryptophan hydroxylase

A

converts tryptophan to 5-hydroxytryptophan
an intermediate in the synthesis of serotonin.
requires BH4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Essential or Nonessential - Glycine

A

Nonessential - glucose

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

how does bilirubin get to the liver

A

it is only slightly water soluble so binds noncovalently to albumin.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

where is fumarate formed in urea cycle?

A

argininosuccinate lyase to convert arginosuccinate into fumarate and arginine.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

which amino acids are degraded into alpha-ketoglutarate

A

Arginine, histidine, glutamine, and proline –> glutamate –> alpha ketoglutarate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Ubiquitination

A

Series of 3 enzymes E1-3 add ubiquitin to lysine (poly ubiquitin) to target protein for degradation by proteosome.
Requires ATP
Proteosome unwinds protein and degrades them into Amino Acids.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

mycophrenolic acid

A

drug that reversibly inhibits GMP formation

prevents T and B cell proliferation and graft cell rejection.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Cytosine Arabinoaside

A

inhibitor of DNA polymerase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

5’ Nucoleotidase

A

removes the phosphate in purine degradation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what is SAM

A

a carbon donor and high energy storage unit.
similar to ATP except that a methyl group replaces the phosphate.
essential for methylation of protein,nucleic acids, lipids,c carbs, epineprine to NE
Epigenetics host defense
Cancer
treats depression.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Bilirubin-Glucuronyl Tranferase

A

facilitates the conjugated of bilirubin in the hepatocyte.
bilirubin –> bilirubin diglucuronide
This is called conjugation
converts UDP glucuronic acid to UDP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

delta-Aminolevulinic acid synthase

A

glycine + Succinyl CoA (from TCA) condensation to form –> Aminolevulinic Acid (ALA)
in Heme Synthesis
requires PLP, rate limiting step of porphyrin biosynthesis
in MITO

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

formation of urogilinogen

A

hydrolyzation of bilirubin diglucoruonide in gut to yeild urobiolinogen.
some is recycled back to enter portal blood.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what is the intermediate fromed in purine synthesis?

A

IMP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Regulation of Urea Cycle

A

1) aminotranaminases with [Prod] & [reactants]
2) Carbomyl phosphate synthase I
3) [Glutamate], [alpha ketoacid] and [NH3]
4) Glutamate dehydrogenase and ATP vs ADP levels

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

R group type - Tryptophan

A

NP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

NO synthase

A

occurs in nerve and muscle
converts Arginine into cirtulline and forms NO in the process
requires tetrahydrofolate and NADPH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

CTP synthetase

A

converts UTP to CTP with addition of glutamine and ATP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

R group type - Lysine

A

Basic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Porphyria

A

rare, inherited defect in heme syntehsis resulting in accumulation of porphyrins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

which portion of urea cycle does asparate enter?

A

arginosuccinate synthase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Essential or Nonessential - Asparagine

A

Non essential - synthesized from glucose

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Pyrimidine synthesis starting materials

A

2ATP
CO2
Glutamine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Pyridoxal phosphate

A

PLP
derivative of Vitamin B6 that serves as coenzyme for aminotransferases.
holds the NH3 during transfer to form a Schiff base - covalent link

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

branched Amino acids

A

Valine, Isoleucine, and leucine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Glutathione

A

modification of amino acids

1) redox buffer to maintain proteins in reduced forms and regulate enzymatic activity
2) cofactor for glutathione transferase
3) reduces H2O2 for protection form ROS
4) proper disulfide bond formation in protein folding
5) facilitates the conversion of methemoglobin into active hemoglobin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Glutathione peroxidase

A

converts GSH to GSSG and also H2O2 to H2O

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

leucine ketogenic or glucogenic

A

ketogenic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Carbomyl phosphate synthase II

A

first step in pyrmidine synthesis
CO2 and glutamine + ATP –> Carbomyl phosphate
occurs in cytosol.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

hydroxyurea inhibits what?

A

UDP –> dUDP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

when is PRPP added in pyrimidine synthesis

A

almost until the end. added to Orotic acid!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

How many ATP are required in Urea Cycle

A

3 ATP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Guanase

A

converts guanine –> xanthene (removes NH3)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Essential or Nonessential - Serine

A

Non essential - synthesized from glucose

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

R group type - tyrosine

A

polar

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Essential or Nonessential - Tryptophan

A

Essential

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Homocystinuria

A

deficiency in cystathione beta synthase to lead to build up of homocysteine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Hydroxylproline

A

post translational modification of Porline by
Prolyl Hydroxase.
Responsible for hydrogen bonding between and within collagen triple strands.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

R group type - Serine

A

polar

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

which amino acids have sulfur

A

only 2
methionine
cysteine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

which amino acids are degraded into fumerate?

A

phenylalanine –> tyrosine

Tyrosine and asparate –> fumerate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Acivicin

A

Gln analog, inhibitor of NT synthesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Essential or Nonessential - Alanine

A

Non essential - synthesized from glucose

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Mutations in glutathione peroxidase

A

increase breast cancer risk

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

What are the components of glutathione?

A

Glutamate + Cysteine + glycine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Pyrimidine breakdown

A

base ring is opened (unlike purine) and products are succinyl coA, Malonyl CoA, Acetyl-CoA.
water soluble.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

R group type - Threonine

A

polar

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

what amino acids are synthesized from TCA cycle

A

Alpha ketoglutarate - Glutamate and glutamine. Glutmate forms proline and arginine.
OAA- asparate and asparigine.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

Isoleucine ketogenic or glucogenic

A

both

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

Thyroid hormone synthesis

A

Tyrosine converted to T4 - thyroxine and T3 Tridothyronine.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

Salvage pathways

A

used to reassemble purine and pyrimidines without havign to undergo denovo synthesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Azidothymidine

A

AZT

inhibitor of Viral Polymerase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

thymidylate synthase

A

converts dUMP into dTMP

requires THF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

Phenylkeotonuria

A

deficiency in phenylalanine hydroxylase to cuase build up of phenylalanine and deficiency in tyrosine.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

R group type - Valine

A

NP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Essential or Nonessential - Valine

A

Essential

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Phenylalanine hydroxylase

A

degradation of pheylalanine to tyrosine.

Requires NADH and BH4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

how is homocysteine converted to cysteine

A

Hcy condenses with SERINE to form cystathionine.

Cystathionine is then converted into cysteine.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

what amino acids are synthesized from glucolysis

A

Serine, Cysteine, glycine,

Alanine (pyruvate)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

reduced form of cysteine

A

single cysteine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

Ornithine transcarboamylase deficiency

A

buildup of ornithine and shortage of citrulline.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

where is ATP required in Glutathione synthesis

A

in both steps - requires 1 ATP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

Adenine Phosphoribosyltransferase

A

purine salvage

converts adenine into AMP with addition of PRPP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

R group type - Cysteine

A

Polar

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

Heme Synthesis

A

occurs in the liver and erythrocyte producing cell of bone marrow
! synthesis rate is variable depending on cellular heme pool in live,r but constant in erythroid cells.
From Glycine and succinyl CoA
combination of in mito (initial rxn and last three steps of porphyrin formation) and cyto

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

Ribonucleotide reductase

A

enzyme that converst ribonucleotides to deoxyribonucleotides.
requires thioredoxin (2SH) to be oxidized to thioredoxin (SS)
inhibited by dNTPs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

how is GABA formed?

A

Glutamate – (Glutamate decarboxylase) –> GABA

requires PLP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

oxidized form of cysteine

A

double bond - disulfide bond.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

Purine Synthesis begins with a…

A

Ribose 5 phosphate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

Transamination

A

transfer of an amino group from an Amino Acid to alpha-ketoglutarate.
Reversible
located in the cytosol as a dimer with two active sites

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

where is the nitrogen input in the urea cycle

A

Ammonia

Asparate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

Homocystinuria

A

defect in cystathionine beta synthase activity with large elevations in homocysteine levels.
associated with mental retardation, osteoporosis, vascular disease (premature thromboci complications before 30 yo)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

Glutamine Phosphoribosyl pyrophosphate amidotransferase

A

PRPP –> 5’-phosphoribosylamine in purine synthesis
Glutamine is added.
Activated by PRPP
inhibited by AMP, GMP, and IMP
the most important regulatory step in purine synthesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

which amino acids are degraded into succinyl Co-A

A

Valine, threonine, isoleucine, methionione –> propionyl-CoA –> Methylmalonyl CoA –> succinyl CoA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

Hydroxylysine

A

post translational modification of lysine by lysyl hydroxase

responsible for covalent crosslinks within collagen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

Valine - ketogenic or glucogenic

A

glucogenic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

Lead and heme synthesis

A

inhibits delta-aminolevulinat dehyratase and ferrochelatase.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

Allopurinol

A

inhibition of xanthine oxidase

treatment of gout (uric acid build up -not water soluble)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

Pepsin

A

Proenzyme: endopeptidase that is activated by Hcl
Aspartic Protease
Leaves after terminal of aromatic Phe, Trp, Try

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

Essential or Nonessential - Proline

A

Non essential - synthesized from glucose

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

Specificity of ubiquitination

A

there is more E3>E2>E1 that leads to specificity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
83
Q

S Adenosylhomocysteine

A

SAH is formed by hydrolysis of SAM and transfer of methyl group to methyl acceptor.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
84
Q

Methotrexate

A

inhibits dUMP –> dTMP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
85
Q

AMP is formed via

A

IMP combining with Aspartic acid to form adenylsuccinate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
86
Q

glutaminase

A
enzyme that converts 
Glutamine + H20 --> glutamate and NH3
found only in hepatocytes
other source of NH3 for urea cycle besides glutamate dehydrogenase
also found in kidney epithelial cells
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
87
Q

hyperhomocysenemia

A

due to low folate, VitB6 and vit B12
promotes oxidative damage, infalmmation, endothelial dysfunction.
vitamin supplementtion reduces these levels.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
88
Q

why are purines and pyrimidines important?

A

1) building blocks for nucleic acids
2) energy in ATP or GTP
3) found in coenzymes, CoA, FAD, NAD, NADP
4) intracellular signaling cAMP, and cGMP
5) activated intermediates like UDP glucose, CDP DAG

89
Q

Gout

A

build up of uric acid due to underexcretion or overactivity of xanthine oxidase.

90
Q

R group type - Glutamine

A

Polar

91
Q

R group type - Histidine

A

Basic

92
Q

Trypsin cleaves ater

A

arginine and lysine

Serine Protease cleaves at C terminal of basic Amino Acids

93
Q

steps of forming ATP from AMP

A

Adnylate kinase and then nucleotide diphosphate kinase

both steps require ATP

94
Q

how is ammonia transferred in the blood?

A

glutamine
this is superior because ammonia is toxic
carries it to kidney for excretion as ammonia in urine
or to liver where it is converted to urea

95
Q

which amino acids are degraded into pyruvate

A

Threonine –> Glycine

Glycine and tryptophan –> alanine, serine, cysteine

96
Q

Maple Syrup Urine Disease

A

Defect in degradation of branched chain amino acids.
Defect in alpha-keotacid dehydrogenase (B1)
to cause buildup of alpha keto acid (especially luecine), but also isoleucine, valine.

97
Q

Cystathione beta synthase

A

condenses homocysteine and serine to form cystathionine
cysteiene synthesis pathway
dependent on PLP

98
Q

R group type - Arginine

A

Basic

99
Q

Aspartic Acid Essential or Nonessential -

A

Non essential - synthesized from glucose

100
Q

Xanthine oxidase

A

Hypoxanthine –> xanthene

xanthene –> uric acid

101
Q

Thioredoxin reducase

A

uses NDPH to convert oxidized from of thioredoxin into reduced form
generates power for ribonulceotide reductase.

102
Q

Lysosomal degradation of protein

A

ATP independent

engulfs extracellular proteins or pathogens to mix with digestive enzymes in lysosomes.

103
Q

how is dophamine formed

A

phenylalanine –> tyrosine –> DOPA –> dopamine via DOPA decarboxylase

104
Q

what happens to bilirubin diglucoronide in hepatocyte?

A

this is conjugated bilirubin - is activated transported across concentration gradient into bile
Dubin johnson is impairment of this transport.

105
Q

y-Glu-Cys + Glycine –?–> Glutathione

A

Glutathione synthase

106
Q

how is Histamine formed?

A

Histidine – (histidine decaroxylase) –> Histamine

requires PLP

107
Q

what is the alternative pathway of tyrosine build up

A

through a number of steps is converted to fumarate and acetoacetate

108
Q

how is Epinephrine formed?

A

phenylalanine –> tyrosine –> DOPA –> dopamine –> NE

–> Epinephrine

109
Q

Carboxypeptidase A cleaves after

A

Ala, Ile, Leu, Vale

Metallocarboxypeptidase: C termianl of hydrophobic

110
Q

adenosine deaminase

A

converts adenosine –> inosine (removes NH3) in purine degradation

111
Q

R group type - Asparate

A

Acidic

112
Q

Which amino acids are both ketogenic and glucogenic?

A

Thyreonine, Isoleucine, tryptophan, pheylalanine, trysoine

113
Q

R group type - methionine

A

NP

114
Q

process for pyrimidine synthesis

A

Glutamine + Co2 –> Carbomyl phosphate + asparate –> carbomyl asparate –> orotic acid + PRPP –> OMP –> UMP

115
Q

hyperinsulinism-hyperammomina syndrome

A

mutation in ATP/GTP binding site of glutamate dehydrogenase.

increase NH3 in the blood.

116
Q

TSH

A

thyroid stimulating hormone stimulates iodide uptake and release of T3 and T4

117
Q

R group type - Glutamate

A

Acidic

118
Q

what is the derivative of melatonin

A

tryptophan

119
Q

R group type - Pheylalanine

A

NP

120
Q

thyroid peroxidase

A

converts iodide to I2. I2 is able to react with thyroglobulin

121
Q

cystathionase

A

converts cystathionine into cysteine, alpha keobutyrate and NH4+
requires PLP
Alpha ketobutyrate is converted to proprionyl Coa and Succinyl coA

122
Q

Types of protein degradation

A

1) ATP dependent ubiquitination

2) Lysosomal pathway

123
Q

Porphyrin

A

a cyclic structure made up for 4 pyroles through methenyl bridges that are produced in the liver
bind to Fe2+

124
Q

what is the fate of fumurate from the urea cycle?

A

fumarate can continue in TCA cycle to form malate and then Oxaloacetate and be undergo transamination for reentry into urea cycle OR gluconeogenesis.

125
Q

Tryptophan metabolism

A

1) alanine –> pyruvate
2) acetylacetyl-CoA
3) Serotonin, melatonin, niacin

126
Q

alternative pathway in phenylalanine degradation

A

build up goes to pheylpyruvate and phenylacetate and phenyllactate.

127
Q

Hypoxanthine guanine phosphoribosyltranferase (HGPRT)

A

purine salvage
converts free base back into GMP or IMP
Guanine back into GMP with addition of PRPP
Hypoxanthine into IMP with PRPP

128
Q

PRPP Synthetase

A

first step in purine synthesis
Ribose-5-phosphate –> 5-Phosphoribosyl-1 pyrophosphate.
activated by Pi
inhibited by purine NTs like AMP, IMP, GMP

129
Q

Chymotrypsin cleaves after

A

trp, Tyr, Phe, Met, Leu

Serine Protease: cleaves at C term inus of aromatic and some hydrophobic

130
Q

Cahill cycle

A

cycle between skeletal muscle and liver.
Glucose is transfered from liver to muscle
Glucose is converted to pyruvate in muscle.
Pyruvate is converted to alanine via ALT
Alanine diffuses through blood back to liver.
Alanine is converted back to pyruvate in liver.
pyruvate can generate more glucose.

131
Q

Vitamin C

A

Ascorbate is a coenzyme
required for the synthesis of hydroxylysine and hydroxyproline
without vitamin C, there is a decrease in collagen strength

132
Q

General pattern of how dNTPs are made and inhibited

A

C –> U/T –> G –> A
dTTP inhibits CDP and UDP
dGTP inhbits dUDP and dGDP
dATP inhibits dCDP, dUDP, dGDP, and dADP

133
Q

Elastase cleaves after

A

Ala, Gly, Ser

134
Q

R group type - Proline

A

NP

135
Q

which amino acids are only ketogenic?

A

Leucine and Lysine

136
Q

Tyrosinemia type 1

A

deficiency in fumarylacetoacetate hydrolase to increase succinylacetone in blood

137
Q

ORNT1

A

transporter in the mitochondria

transports cirtulline outside the mitochondria and ornithine inside

138
Q

how is NO formed?

A

Arginine – (NO synthase) –> Citrulline

requires NADPH, BH4

139
Q

Schiff base

A

formed as the intermediate during aminotransferases with PLP.
a covalent bond

140
Q

Creation of serotonin

A

from tryptophan –> 5- hydroxytryptophan –> serotonin.

141
Q

Alanine Aminotransferase

A

facilitates the conversion of Alanine and alphaketoglutarate to pyruvate and glutamate.
ALT
dependent on Pyridoxal Phosphate (PLP)

142
Q

Carbomyl Phosphate Synthase I

A

enzyme in Urea Cycle inside mitochondria
Facilitates NH3 + CO2 –> Carbomoyl Phosphate.
requires 2 ATP (2 of the 3 in the urea cycle)
Regulated by N-Acetylglutamate (activated)

143
Q

IMP forms…

A

AMP and GMP

144
Q

Essential or Nonessential - Leucine

A

Essential

145
Q

how is SAM synthesized

A

Condensation of methionine and ATP via SAM synthase

SAM - S- adenosyl methionine.

146
Q

y-Carboxyglutamate (GLA)

A

post translational modification of glutamate
by gamma-glutamyl carboxylase
formation of two carbonyl groups to facilitate calcium chelation.
Acts as the N terminal of prothrombin that is embedded in the membrane.

147
Q

which are pyrimidines

A

thyamine, cytosine, uracil

148
Q

Tyrosinemia type 2

A

Phenylpyruvic acid dehydrogenase deficiency to increase tyrosine in serum

149
Q

Glutathione, is it reduced or oxidized?

A

GSH reduced form

GSSG is oxidized

150
Q

Essential or Nonessential - Histidine

A

Essential

151
Q

how is homocysteine converted to methionine

A

Homocysteine accepts a methyl group form N5methyl THF
requires Vitamin B12
enzyme: methionine synthase

152
Q

Essential or Nonessential - Methionine

A

Essential

153
Q

N-Actylglutamate

A

product of Acetyl CoA and Glutamate via N.Actylglutamate synthase.
activator of Carbomyl Phosphate Synthase I

154
Q

Purine Degradation

A

AMP degradtion leads to formation of insoine which is converted into hypoxanthine. Hypoxanthine must be converted into xanthene via xanthine oxidase which creates H2O2.
GMP degration does not go through hypoxanthene but instead directly to xanthene.
Xanthene is converted to uric acid via xanthine oxidase again to generate for H2O2.

155
Q

which are purines

A

adenine and guanine

156
Q

Purine degradation first initial steps

A

AMP –(AMP deaminase)–> IMP –(5’nucleotidase) –> Inosine – (purine nucleoside phosphorylase) –> hypoxanthine – (xanthine oxidase)–> xanthene
so removal of amine before removal of phosphate, then removal or ribose
alterantively can be AMP –> adenosine –(adenosine deaminase) –> Inosine
GMP –(5’ nucleotidase) –> Guanosine –(purine nucleoside phsophylase) –> guanine – (guanase)–> xanthene
so removal of phosphate, removal of ribose, then removal of amino

157
Q

how is NE formed?

A

phenylalanine –> tyrosine –> DOPA –> dopamine –> NE

via Dopamine beta hydroxylase

158
Q

Essential or Nonessential - Tyrosine

A

Non essential - synthesized from phenylalanine

159
Q

Amidinotransferase Methyltransferase

A

an alternative route in Urea cycle

adds Glycine into Arginine to form creatine phosphate during the conversion to ornithine.

160
Q

Deiodinase

A

Converts T4 - thyroxine to T3 triiodothyronine.

Tyroxine is a prohormone

161
Q

Bilirubin Glucuonyl Tranferase dysfunction

A

seen in Crigler Najjar Syndrome
Gilbert Syndrome
premature infants

162
Q

Purine nucleoside phosphorylase

A

removes the ribose in purine degradation

163
Q

what is entry point into the urea cycle

A

Free Ammonia as a result of Glutamase dehydrogenase and Asparate from AST

164
Q

Porphobilinogen –>

A

protophorphyrin –> heme (Heme synthesis)
via ferrochelatase
also inhibited by lead
occurs in Mito again

165
Q

how is transamination regulated?

A

by concentration of products and reactants

166
Q

Asparate Aminotransferase

A

Facilitates the reversible converstion of Glutamate and Oxaloacetate to Asparate and alpha Ketoglutarate.
AST
dependent on PLP

167
Q

Essential or Nonessential - Cysteine

A

Nonessential - glucose

168
Q

where is urea produced in urea cycle?

A

via Arginase in conversion of arginine into ornithine.

169
Q

R group type - Isoleucine

A

NP

170
Q

homocysteine methyltransferase

A

adds methyl group onto tetrahydrofluroide.

depends on vitamin B12

171
Q

Glutamine Essential or Nonessential -

A

Non essential - synthesized from glucose

172
Q

5 flurouracil

A

inhibits dUMP –> dTMP

173
Q

Gluconeogenic side of tryptophan metabolism

A

through multiple steps tryptophan is converted to alanine. Alanine undergoes alanine aminotransferase into pyruvate (also alphaketoglutarate into glutamate). Pytruate can enter TCA cycle.

174
Q

what oxidizes glutathione?

A

Glutathione peroxidase

175
Q

Heme oxygenase

A
heme degradation
from Heme --> biliverdin
requires NADPH
and release Fe3+ and CO
occurs in macrophage
176
Q

dTTP syntehsis

A

UMP –> UDP –> dUDP –> dUMP –> dTMP –> dTDP –> dTTP

alternative route; dCDP –> cCMP –> dUMP –> dTMP –> dTDP –> dTTP

177
Q

what are the sources of atoms for purine synthesis

A
Glutamine
Glycine
THF
Asparate
CO2
2ATP
178
Q

Arginase

A

enzyme in urea cycle cytoplasm
only really found in liver
converts arginine into ornithine and produces UREA
inhibited by Ornithine concentrations

179
Q

Ornithine Transcarbamylase

A

Facilitates the joining of Ornithine with Carbomyl Phosphate to form Citrulline.
Release of Phosphate.
Occurs in mitochondria of the Urea Cycle

180
Q

what is the most important step in purine synthesis

A

Glutamine phosphoribosyl pyrophosphate amidotransferase

181
Q

Where is the carbon input in the urea cycle?

A

CO2

182
Q

Where is tyrosine derived from

A

phenylalanine

183
Q

6 mercaptopurine

A

drug that inhibits the formation of IMP in purine synthesis

184
Q

Tyrosine hydroxylase

A

converts tyrosine into DOPA
requires BH4
starts the pathway to create dopamine, norepinephrine, and epinephrine, and melanin.

185
Q

R group type - Asparagine

A

polar

186
Q

Bh4

A

tetrahydrobiopterin cofactor

187
Q

Cyseinuria

A

kidney stones due to defective transportion of cysteine, ornithine, lysine, or arginine.
tx: acetazolamide to increase cysteine solubility.

188
Q

Acycolvir

A

viral DNA polymerase and reverse transcriptase inhibtior

189
Q

what enzymes are in the mitochondria of the Urea Cycle?

A

Carbomyl Phosphate Synthase I

Ornithine transcarbomylase

190
Q

Threonine Essential or Nonessential -

A

Essential

191
Q

Arginosuccinate Lyase

A

urea cycle enzyme in cytoplasm

converts arginosuccinate into arginine and fumarate

192
Q

Essential or Nonessential - Arginine

A

Non essential - synthesized from glucose

193
Q

Stercobilin

A

oxidation of urobilinogen by intestinal bacteria to give feces the brown color.

194
Q

Glutamate dehydrogenase

A

facilitates conversion of glutamate into alpha ketoglutarate and NH3
found in liver, muscle, kidney, and peripheral tissue
requires NADPH
Activated by ADP and GDP
inhibited by ATP and GTP

195
Q

Homocysteine

A
formed when SAH is hydrolyzed by SAH Hydrolyase
releases a adenosine.
Hcy - there are two fates
1) remyelinated back to methionine 
2) transulfuration to cysteine
196
Q

what reduces GSSG?

A

Glutathione reductase and also NADPH to NADP+

197
Q

Glutamate + cysteine –?–> y- Glut-Gys

A

y-glutamyl cysteine synthetase

198
Q

tetrahydrofolate

A

produced from Folic acid (vitamin B9) via Dihydrofolate reducatase (DHFR)

199
Q

Bilverdin Redutase

A

converts bilverdin to bilirubin in heme degradation.
requires NADPH
occurs in macrophage

200
Q

Thyroglobulin

A

a huge 660 kDa protein with 140 tyrosines that are iodinated and protealyzed to make T3 and T4.
T3 and T4 are transferred in blood via Thryoxine binding globulin.

201
Q

Pyrimidine Synthesis begins with a…

A

Base (Glutamine)

202
Q

dela - ammolevulinate dehydratase

A

converts aminolevulinic acid (ALA) into prophobilinogen in cytoplasm
zinc dependent
inhibited by lead

203
Q

Phenylalanine –?–> tyrosine

A

phenylalanine hydroxylase

204
Q

Essential or Nonessential - Lysine

A

Essential

205
Q

R group type - Leucine

A

NP

206
Q

which amino acids are degraded into oxaloacetate?

A

asparate and asparagine

207
Q

how do ribonucleotides get made into deoxyribonucleotides?

A

via ribonucleotide reductase

208
Q

Essential or Nonessential - Isoleucine

A

Essential

209
Q

dCTP sythesis

A

UMP –> UDP –> UTP –> CTP –> CDP –> dCDP –> dCTP

210
Q

Argininosuccinate Synthase

A

Urea Cycle Enzyme in cytoplasm
Facilitates Citrilline and asparate to form arginosuccinate.
requires 1 ATP

211
Q

Dubin Johnson

A

mutation in bilirubin transporter MRP2 out of hepatocyte into bile

212
Q

Adenosine deaminase deficiency

A

severe combined immunodeficiency syndrome
leads to a buildup of ATP and dATP, to inhibit ribonucleotide reducatase to decrease dNTP synthesis.
can no longer make rapidly proliferating Lymphocytes

213
Q

Carboxypeptidase B cleaves after

A

Arg, Lys

Metallocarboxypeptidase: C terminal of basic AA

214
Q

OMP forms

A

UMP –> UDP –> UTP -> CTP

215
Q

Lesh Nyhan Syndrome

A

X linked recessive
deficiency of hypoxanthine guanine phosphoribosyltranferase (HGPRT)
so can’t salvage hypoxanthine or guanine.
buildup of PRPP and decrease IMP and GMP
so increased Uric acid, self mutilation, mental disorders
Txwith allopurinol, and febuxostat

216
Q

R group type - Alanine

A

NP

217
Q

Essential or Nonessential - Glutamic acid

A

Non essential - synthesized from glucose

218
Q

what is the derivative of melanin

A

DOPA and tyrosine (phenylalanine)

219
Q

R group type - Glycine

A

NP