Protein- Metabolism Flashcards
1
Q
Essential or Nonessential - Phenylalanine
A
Essential
2
Q
Tryptophan hydroxylase
A
converts tryptophan to 5-hydroxytryptophan
an intermediate in the synthesis of serotonin.
requires BH4
3
Q
Essential or Nonessential - Glycine
A
Nonessential - glucose
4
Q
how does bilirubin get to the liver
A
it is only slightly water soluble so binds noncovalently to albumin.
5
Q
where is fumarate formed in urea cycle?
A
argininosuccinate lyase to convert arginosuccinate into fumarate and arginine.
6
Q
which amino acids are degraded into alpha-ketoglutarate
A
Arginine, histidine, glutamine, and proline –> glutamate –> alpha ketoglutarate
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.
8
Q
mycophrenolic acid
A
drug that reversibly inhibits GMP formation
prevents T and B cell proliferation and graft cell rejection.
9
Q
Cytosine Arabinoaside
A
inhibitor of DNA polymerase
10
Q
5’ Nucoleotidase
A
removes the phosphate in purine degradation
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.
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
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
14
Q
formation of urogilinogen
A
hydrolyzation of bilirubin diglucoruonide in gut to yeild urobiolinogen.
some is recycled back to enter portal blood.
15
Q
what is the intermediate fromed in purine synthesis?
A
IMP
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
17
Q
R group type - Tryptophan
A
NP
18
Q
NO synthase
A
occurs in nerve and muscle
converts Arginine into cirtulline and forms NO in the process
requires tetrahydrofolate and NADPH
19
Q
CTP synthetase
A
converts UTP to CTP with addition of glutamine and ATP
20
Q
R group type - Lysine
A
Basic
21
Q
Porphyria
A
rare, inherited defect in heme syntehsis resulting in accumulation of porphyrins
22
Q
which portion of urea cycle does asparate enter?
A
arginosuccinate synthase
23
Q
Essential or Nonessential - Asparagine
A
Non essential - synthesized from glucose
24
Q
Pyrimidine synthesis starting materials
A
2ATP
CO2
Glutamine
25
Pyridoxal phosphate
PLP
derivative of Vitamin B6 that serves as coenzyme for aminotransferases.
holds the NH3 during transfer to form a Schiff base - covalent link
26
branched Amino acids
Valine, Isoleucine, and leucine
27
Glutathione
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
28
Glutathione peroxidase
converts GSH to GSSG and also H2O2 to H2O
29
leucine ketogenic or glucogenic
ketogenic
30
Carbomyl phosphate synthase II
first step in pyrmidine synthesis
CO2 and glutamine + ATP --> Carbomyl phosphate
occurs in cytosol.
31
hydroxyurea inhibits what?
UDP --> dUDP
32
when is PRPP added in pyrimidine synthesis
almost until the end. added to Orotic acid!
33
How many ATP are required in Urea Cycle
3 ATP
34
Guanase
converts guanine --> xanthene (removes NH3)
35
Essential or Nonessential - Serine
Non essential - synthesized from glucose
36
R group type - tyrosine
polar
37
Essential or Nonessential - Tryptophan
Essential
38
Homocystinuria
deficiency in cystathione beta synthase to lead to build up of homocysteine
39
Hydroxylproline
post translational modification of Porline by
Prolyl Hydroxase.
Responsible for hydrogen bonding between and within collagen triple strands.
40
R group type - Serine
polar
41
which amino acids have sulfur
only 2
methionine
cysteine
42
which amino acids are degraded into fumerate?
phenylalanine --> tyrosine
| Tyrosine and asparate --> fumerate
43
Acivicin
Gln analog, inhibitor of NT synthesis
44
Essential or Nonessential - Alanine
Non essential - synthesized from glucose
45
Mutations in glutathione peroxidase
increase breast cancer risk
46
What are the components of glutathione?
Glutamate + Cysteine + glycine
47
Pyrimidine breakdown
base ring is opened (unlike purine) and products are succinyl coA, Malonyl CoA, Acetyl-CoA.
water soluble.
48
R group type - Threonine
polar
49
what amino acids are synthesized from TCA cycle
Alpha ketoglutarate - Glutamate and glutamine. Glutmate forms proline and arginine.
OAA- asparate and asparigine.
50
Isoleucine ketogenic or glucogenic
both
51
Thyroid hormone synthesis
Tyrosine converted to T4 - thyroxine and T3 Tridothyronine.
52
Salvage pathways
used to reassemble purine and pyrimidines without havign to undergo denovo synthesis
53
Azidothymidine
AZT
| inhibitor of Viral Polymerase
54
thymidylate synthase
converts dUMP into dTMP
| requires THF
55
Phenylkeotonuria
deficiency in phenylalanine hydroxylase to cuase build up of phenylalanine and deficiency in tyrosine.
56
R group type - Valine
NP
57
Essential or Nonessential - Valine
Essential
58
Phenylalanine hydroxylase
degradation of pheylalanine to tyrosine.
| Requires NADH and BH4
59
how is homocysteine converted to cysteine
Hcy condenses with SERINE to form cystathionine.
| Cystathionine is then converted into cysteine.
60
what amino acids are synthesized from glucolysis
Serine, Cysteine, glycine,
| Alanine (pyruvate)
61
reduced form of cysteine
single cysteine
62
Ornithine transcarboamylase deficiency
buildup of ornithine and shortage of citrulline.
63
where is ATP required in Glutathione synthesis
in both steps - requires 1 ATP
64
Adenine Phosphoribosyltransferase
purine salvage
| converts adenine into AMP with addition of PRPP
65
R group type - Cysteine
Polar
66
Heme Synthesis
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
67
Ribonucleotide reductase
enzyme that converst ribonucleotides to deoxyribonucleotides.
requires thioredoxin (2SH) to be oxidized to thioredoxin (SS)
inhibited by dNTPs
68
how is GABA formed?
Glutamate -- (Glutamate decarboxylase) --> GABA
| requires PLP
69
oxidized form of cysteine
double bond - disulfide bond.
70
Purine Synthesis begins with a...
Ribose 5 phosphate
71
Transamination
transfer of an amino group from an Amino Acid to alpha-ketoglutarate.
Reversible
located in the cytosol as a dimer with two active sites
72
where is the nitrogen input in the urea cycle
Ammonia
| Asparate
73
Homocystinuria
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)
74
Glutamine Phosphoribosyl pyrophosphate amidotransferase
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
75
which amino acids are degraded into succinyl Co-A
Valine, threonine, isoleucine, methionione --> propionyl-CoA --> Methylmalonyl CoA --> succinyl CoA
76
Hydroxylysine
post translational modification of lysine by lysyl hydroxase
| responsible for covalent crosslinks within collagen
77
Valine - ketogenic or glucogenic
glucogenic
78
Lead and heme synthesis
inhibits delta-aminolevulinat dehyratase and ferrochelatase.
79
Allopurinol
inhibition of xanthine oxidase
| treatment of gout (uric acid build up -not water soluble)
80
Pepsin
Proenzyme: endopeptidase that is activated by Hcl
Aspartic Protease
Leaves after terminal of aromatic Phe, Trp, Try
81
Essential or Nonessential - Proline
Non essential - synthesized from glucose
82
Specificity of ubiquitination
there is more E3>E2>E1 that leads to specificity
83
S Adenosylhomocysteine
SAH is formed by hydrolysis of SAM and transfer of methyl group to methyl acceptor.
84
Methotrexate
inhibits dUMP --> dTMP
85
AMP is formed via
IMP combining with Aspartic acid to form adenylsuccinate
86
glutaminase
```
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
```
87
hyperhomocysenemia
due to low folate, VitB6 and vit B12
promotes oxidative damage, infalmmation, endothelial dysfunction.
vitamin supplementtion reduces these levels.
88
why are purines and pyrimidines important?
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
Gout
build up of uric acid due to underexcretion or overactivity of xanthine oxidase.
90
R group type - Glutamine
Polar
91
R group type - Histidine
Basic
92
Trypsin cleaves ater
arginine and lysine
| Serine Protease cleaves at C terminal of basic Amino Acids
93
steps of forming ATP from AMP
Adnylate kinase and then nucleotide diphosphate kinase
| both steps require ATP
94
how is ammonia transferred in the blood?
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
which amino acids are degraded into pyruvate
Threonine --> Glycine
| Glycine and tryptophan --> alanine, serine, cysteine
96
Maple Syrup Urine Disease
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
Cystathione beta synthase
condenses homocysteine and serine to form cystathionine
cysteiene synthesis pathway
dependent on PLP
98
R group type - Arginine
Basic
99
Aspartic Acid Essential or Nonessential -
Non essential - synthesized from glucose
100
Xanthine oxidase
Hypoxanthine --> xanthene
| xanthene --> uric acid
101
Thioredoxin reducase
uses NDPH to convert oxidized from of thioredoxin into reduced form
generates power for ribonulceotide reductase.
102
Lysosomal degradation of protein
ATP independent
| engulfs extracellular proteins or pathogens to mix with digestive enzymes in lysosomes.
103
how is dophamine formed
phenylalanine --> tyrosine --> DOPA --> dopamine via DOPA decarboxylase
104
what happens to bilirubin diglucoronide in hepatocyte?
this is conjugated bilirubin - is activated transported across concentration gradient into bile
Dubin johnson is impairment of this transport.
105
y-Glu-Cys + Glycine --?--> Glutathione
Glutathione synthase
106
how is Histamine formed?
Histidine -- (histidine decaroxylase) --> Histamine
| requires PLP
107
what is the alternative pathway of tyrosine build up
through a number of steps is converted to fumarate and acetoacetate
108
how is Epinephrine formed?
phenylalanine --> tyrosine --> DOPA --> dopamine --> NE
| --> Epinephrine
109
Carboxypeptidase A cleaves after
Ala, Ile, Leu, Vale
| Metallocarboxypeptidase: C termianl of hydrophobic
110
adenosine deaminase
converts adenosine --> inosine (removes NH3) in purine degradation
111
R group type - Asparate
Acidic
112
Which amino acids are both ketogenic and glucogenic?
Thyreonine, Isoleucine, tryptophan, pheylalanine, trysoine
113
R group type - methionine
NP
114
process for pyrimidine synthesis
Glutamine + Co2 --> Carbomyl phosphate + asparate --> carbomyl asparate --> orotic acid + PRPP --> OMP --> UMP
115
hyperinsulinism-hyperammomina syndrome
mutation in ATP/GTP binding site of glutamate dehydrogenase.
| increase NH3 in the blood.
116
TSH
thyroid stimulating hormone stimulates iodide uptake and release of T3 and T4
117
R group type - Glutamate
Acidic
118
what is the derivative of melatonin
tryptophan
119
R group type - Pheylalanine
NP
120
thyroid peroxidase
converts iodide to I2. I2 is able to react with thyroglobulin
121
cystathionase
converts cystathionine into cysteine, alpha keobutyrate and NH4+
requires PLP
Alpha ketobutyrate is converted to proprionyl Coa and Succinyl coA
122
Types of protein degradation
1) ATP dependent ubiquitination
| 2) Lysosomal pathway
123
Porphyrin
a cyclic structure made up for 4 pyroles through methenyl bridges that are produced in the liver
bind to Fe2+
124
what is the fate of fumurate from the urea cycle?
fumarate can continue in TCA cycle to form malate and then Oxaloacetate and be undergo transamination for reentry into urea cycle OR gluconeogenesis.
125
Tryptophan metabolism
1) alanine --> pyruvate
2) acetylacetyl-CoA
3) Serotonin, melatonin, niacin
126
alternative pathway in phenylalanine degradation
build up goes to pheylpyruvate and phenylacetate and phenyllactate.
127
Hypoxanthine guanine phosphoribosyltranferase (HGPRT)
purine salvage
converts free base back into GMP or IMP
Guanine back into GMP with addition of PRPP
Hypoxanthine into IMP with PRPP
128
PRPP Synthetase
first step in purine synthesis
Ribose-5-phosphate --> 5-Phosphoribosyl-1 pyrophosphate.
activated by Pi
inhibited by purine NTs like AMP, IMP, GMP
129
Chymotrypsin cleaves after
trp, Tyr, Phe, Met, Leu
| Serine Protease: cleaves at C term inus of aromatic and some hydrophobic
130
Cahill cycle
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
Vitamin C
Ascorbate is a coenzyme
required for the synthesis of hydroxylysine and hydroxyproline
without vitamin C, there is a decrease in collagen strength
132
General pattern of how dNTPs are made and inhibited
C --> U/T --> G --> A
dTTP inhibits CDP and UDP
dGTP inhbits dUDP and dGDP
dATP inhibits dCDP, dUDP, dGDP, and dADP
133
Elastase cleaves after
Ala, Gly, Ser
134
R group type - Proline
NP
135
which amino acids are only ketogenic?
Leucine and Lysine
136
Tyrosinemia type 1
deficiency in fumarylacetoacetate hydrolase to increase succinylacetone in blood
137
ORNT1
transporter in the mitochondria
| transports cirtulline outside the mitochondria and ornithine inside
138
how is NO formed?
Arginine -- (NO synthase) --> Citrulline
| requires NADPH, BH4
139
Schiff base
formed as the intermediate during aminotransferases with PLP.
a covalent bond
140
Creation of serotonin
from tryptophan --> 5- hydroxytryptophan --> serotonin.
141
Alanine Aminotransferase
facilitates the conversion of Alanine and alphaketoglutarate to pyruvate and glutamate.
ALT
dependent on Pyridoxal Phosphate (PLP)
142
Carbomyl Phosphate Synthase I
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
IMP forms...
AMP and GMP
144
Essential or Nonessential - Leucine
Essential
145
how is SAM synthesized
Condensation of methionine and ATP via SAM synthase
| SAM - S- adenosyl methionine.
146
y-Carboxyglutamate (GLA)
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
which are pyrimidines
thyamine, cytosine, uracil
148
Tyrosinemia type 2
Phenylpyruvic acid dehydrogenase deficiency to increase tyrosine in serum
149
Glutathione, is it reduced or oxidized?
GSH reduced form
| GSSG is oxidized
150
Essential or Nonessential - Histidine
Essential
151
how is homocysteine converted to methionine
Homocysteine accepts a methyl group form N5methyl THF
requires Vitamin B12
enzyme: methionine synthase
152
Essential or Nonessential - Methionine
Essential
153
N-Actylglutamate
product of Acetyl CoA and Glutamate via N.Actylglutamate synthase.
activator of Carbomyl Phosphate Synthase I
154
Purine Degradation
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
which are purines
adenine and guanine
156
Purine degradation first initial steps
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
how is NE formed?
phenylalanine --> tyrosine --> DOPA --> dopamine --> NE
| via Dopamine beta hydroxylase
158
Essential or Nonessential - Tyrosine
Non essential - synthesized from phenylalanine
159
Amidinotransferase Methyltransferase
an alternative route in Urea cycle
| adds Glycine into Arginine to form creatine phosphate during the conversion to ornithine.
160
Deiodinase
Converts T4 - thyroxine to T3 triiodothyronine.
| Tyroxine is a prohormone
161
Bilirubin Glucuonyl Tranferase dysfunction
seen in Crigler Najjar Syndrome
Gilbert Syndrome
premature infants
162
Purine nucleoside phosphorylase
removes the ribose in purine degradation
163
what is entry point into the urea cycle
Free Ammonia as a result of Glutamase dehydrogenase and Asparate from AST
164
Porphobilinogen -->
protophorphyrin --> heme (Heme synthesis)
via ferrochelatase
also inhibited by lead
occurs in Mito again
165
how is transamination regulated?
by concentration of products and reactants
166
Asparate Aminotransferase
Facilitates the reversible converstion of Glutamate and Oxaloacetate to Asparate and alpha Ketoglutarate.
AST
dependent on PLP
167
Essential or Nonessential - Cysteine
Nonessential - glucose
168
where is urea produced in urea cycle?
via Arginase in conversion of arginine into ornithine.
169
R group type - Isoleucine
NP
170
homocysteine methyltransferase
adds methyl group onto tetrahydrofluroide.
| depends on vitamin B12
171
Glutamine Essential or Nonessential -
Non essential - synthesized from glucose
172
5 flurouracil
inhibits dUMP --> dTMP
173
Gluconeogenic side of tryptophan metabolism
through multiple steps tryptophan is converted to alanine. Alanine undergoes alanine aminotransferase into pyruvate (also alphaketoglutarate into glutamate). Pytruate can enter TCA cycle.
174
what oxidizes glutathione?
Glutathione peroxidase
175
Heme oxygenase
```
heme degradation
from Heme --> biliverdin
requires NADPH
and release Fe3+ and CO
occurs in macrophage
```
176
dTTP syntehsis
UMP --> UDP --> dUDP --> dUMP --> dTMP --> dTDP --> dTTP
| alternative route; dCDP --> cCMP --> dUMP --> dTMP --> dTDP --> dTTP
177
what are the sources of atoms for purine synthesis
```
Glutamine
Glycine
THF
Asparate
CO2
2ATP
```
178
Arginase
enzyme in urea cycle cytoplasm
only really found in liver
converts arginine into ornithine and produces UREA
inhibited by Ornithine concentrations
179
Ornithine Transcarbamylase
Facilitates the joining of Ornithine with Carbomyl Phosphate to form Citrulline.
Release of Phosphate.
Occurs in mitochondria of the Urea Cycle
180
what is the most important step in purine synthesis
Glutamine phosphoribosyl pyrophosphate amidotransferase
181
Where is the carbon input in the urea cycle?
CO2
182
Where is tyrosine derived from
phenylalanine
183
6 mercaptopurine
drug that inhibits the formation of IMP in purine synthesis
184
Tyrosine hydroxylase
converts tyrosine into DOPA
requires BH4
starts the pathway to create dopamine, norepinephrine, and epinephrine, and melanin.
185
R group type - Asparagine
polar
186
Bh4
tetrahydrobiopterin cofactor
187
Cyseinuria
kidney stones due to defective transportion of cysteine, ornithine, lysine, or arginine.
tx: acetazolamide to increase cysteine solubility.
188
Acycolvir
viral DNA polymerase and reverse transcriptase inhibtior
189
what enzymes are in the mitochondria of the Urea Cycle?
Carbomyl Phosphate Synthase I
| Ornithine transcarbomylase
190
Threonine Essential or Nonessential -
Essential
191
Arginosuccinate Lyase
urea cycle enzyme in cytoplasm
| converts arginosuccinate into arginine and fumarate
192
Essential or Nonessential - Arginine
Non essential - synthesized from glucose
193
Stercobilin
oxidation of urobilinogen by intestinal bacteria to give feces the brown color.
194
Glutamate dehydrogenase
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
Homocysteine
```
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
what reduces GSSG?
Glutathione reductase and also NADPH to NADP+
197
Glutamate + cysteine --?--> y- Glut-Gys
y-glutamyl cysteine synthetase
198
tetrahydrofolate
produced from Folic acid (vitamin B9) via Dihydrofolate reducatase (DHFR)
199
Bilverdin Redutase
converts bilverdin to bilirubin in heme degradation.
requires NADPH
occurs in macrophage
200
Thyroglobulin
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
Pyrimidine Synthesis begins with a...
Base (Glutamine)
202
dela - ammolevulinate dehydratase
converts aminolevulinic acid (ALA) into prophobilinogen in cytoplasm
zinc dependent
inhibited by lead
203
Phenylalanine --?--> tyrosine
phenylalanine hydroxylase
204
Essential or Nonessential - Lysine
Essential
205
R group type - Leucine
NP
206
which amino acids are degraded into oxaloacetate?
asparate and asparagine
207
how do ribonucleotides get made into deoxyribonucleotides?
via ribonucleotide reductase
208
Essential or Nonessential - Isoleucine
Essential
209
dCTP sythesis
UMP --> UDP --> UTP --> CTP --> CDP --> dCDP --> dCTP
210
Argininosuccinate Synthase
Urea Cycle Enzyme in cytoplasm
Facilitates Citrilline and asparate to form arginosuccinate.
requires 1 ATP
211
Dubin Johnson
mutation in bilirubin transporter MRP2 out of hepatocyte into bile
212
Adenosine deaminase deficiency
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
Carboxypeptidase B cleaves after
Arg, Lys
| Metallocarboxypeptidase: C terminal of basic AA
214
OMP forms
UMP --> UDP --> UTP -> CTP
215
Lesh Nyhan Syndrome
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
R group type - Alanine
NP
217
Essential or Nonessential - Glutamic acid
Non essential - synthesized from glucose
218
what is the derivative of melanin
DOPA and tyrosine (phenylalanine)
219
R group type - Glycine
NP
