Nitrogen Metabolism, AA metab, Urea cycle, AA dx, Heme metab and Integration of Metabolism Flashcards
(270 cards)
1
Q
Net accumulation of proteins as in growth and pregnancy
A
Positive nitrogen balance
2
Q
Net breakdown of proteins as in surgery, advanced cancer, kwashiorkor or marasmus, starvation
A
Negative nitrogen balance
3
Q
Average protein turnover per day
A
300 to 400 g per day
4
Q
Amount of protein degraded and resynthesized from AA
A
Protein turnover
5
Q
Sum of all free amino acids in cells and ECF
A
Amino acid pool
6
Q
First phase of amino acid catabolism:
Removal of alpha amino group, forming ammonia and alpha keto acid
A
Deamination
7
Q
First phase of amino acid catabolism:
Removal of amino group for AA yields these 2 products
A
Alpha keto acid
Ammonia
8
Q
First phase of amino acid catabolism:
Ammonia may be excreted as free ammonia in urine and stool, but majority is converted to _____
A
Urea
9
Q
Second phase of AA catabolism
Carbon skeletons of alpha keto acids are converted to common intermediates of what 2 energy-producing metabolic pathways
A
Glycolysis
TCA
10
Q
Term that denotes excretion of nitrogen among telostean fish, which excrete highly toxic ammonia
A
Ammonotelic
11
Q
Term that denotes manner of excretion of excess nitrogen among birds, which excrete uric acid as semisolid guano
A
Uricotelic
12
Q
Term that denotes manner of excretion of excess nitrogen among land animals including humans, who excrete non-toxic, water-soluble urea
A
Ureotelic
13
Q
Amino acids transfer their amino group to a-kg resulting in the formation glutamate
A
Transamination
14
Q
During transamination, amino acids transfer their amino group to a-kg resulting in the formation of _____
A
Glutamate
15
Q
Enzyme for transamination
A
Aminot transferase
16
Q
Co enzyme of aminotransferase
A
Pyridoxal phosphate
17
Q
Alanine + a-kg —> _____ + ______
A
Glutamate, pyruvate
18
Q
Aspartate + a-kg —> ____ + _____
A
Glutamate, OAA
19
Q
2 major step in the first phase of AA catabolism
A
Transamination
Oxidative deamination
20
Q
AA catabolism
In the liver and kidney, glutamate is oxidatively deaminated to release free ammonia
A
Oxidative deamination
21
Q
2 organs involved in oxidative deamination
A
Liver
Kidney
22
Q
Enzyme for oxidative deamination
A
Glutamate dehydrogenase
23
Q
AA catabolism
In the liver and kidney, _______ is oxidatively deaminated to release free ammonia
A
Glutamate
24
Q
AA catabolism
In the liver and kidney, glutamate is oxidatively deaminated to release _____
A
Free ammonia
25
2 AA that are used for transport of ammonia from peripheral tissues
Glutamine
| Alanine
26
TRANSPORT OF AMMONIA FROM PERIPHERAL TISSUES
| In muscle, pyruvate is transaminated to _____
Alanine
27
Alanine is transported to the liver where it is converted back to ______
Pyruvate
28
In most tissues, glutamate combines with ammonia to form ____ via the enzyme ______
Glutamine
| Glutamine synthetase
29
Glutamine is transported in the blood and may be deaminated to release ammonia in two organs:
Liver
| Kidneys
30
Liver releases ammonia in response to _____
High protein intake
31
Kidneys release ammonia in response to ______
Metabolic acidosis
32
Pathway for the conversion of the body’s nitrogenous waste to urea
Urea cycle
33
Organ site of urea cycle
Liver
34
Subcellular site of urea cycle
Mitochondria and cytosol
35
3 substrates for urea cycle
CO2, ammonia, aspartate
36
Product of urea cycle
Urea
37
Rate-limiting step for urea cycle
NH3 + CO2 —> carbamoyl phosphate
| Enzyme: carbamoyl phosphate synthetase I
38
Allosteric activator for the rate-limiting step of urea cycle
N-acetylglutamate
39
Identify enzyme for this step in the urea cycle
| CO2 + NH3 —> carbamoyl phosphate
Carbamoyl phosphate synthetase I
40
Identify enzyme for this step in the urea cycle
| Carbamoyl phosphate + L-ornithine —> citrulline
Ornithine transcarbamoylase
41
Identify enzyme for this step in the urea cycle
| Citrulline + aspartate —> argininosuccinate
Argininosuccinate synthetase
42
Identify enzyme for this step in the urea cycle
| Argininosuccinate —> fumarate
Argininosuccinate lyase
43
Identify enzyme for this step in the urea cycle
| L-arginine —> urea + L-ornithine
Arginase
44
Identify products in this step of urea cycle
| Carbamoyl phosphate + L ornithine —>
Citrulline
45
Identify products in this step of urea cycle
| Citrulline + aspartate —>
Argininosuccinate
46
Identify products in this step of urea cycle
| Argininosuccinate —>
Fumarate + L-arginine
47
Identify products in this step of urea cycle
| L-arginine + H20 —>
Urea, L-ornithine
48
2 reactions in urea cycle that require ATP
CO2 + NH3 —> carbamoyl phosphate (2 ATPs)
| Citrulline + L-aspartate —> argininosuccinate (1 ATP)
49
How many ATPs are required to form 1 mol of urea?
3 (but 4 high energy bonds)
50
T/F
| In urea cycle, there is no net loss or gain of ornithine, citrulline, argininosuccinate, or arginine
T
51
_______ functions solely as enzyme activator and regulates urea synthesis
N-acetylglutamate
52
2 fates of urea:
Blood—>kidney —> urine
| Intestines—> urease converting bacteria convert them back to CO2 and NH3
53
Tremors, slurring of speech, vomiting, blurring if vision
Cerebral edema
Somnolence, coma, death
Hyperammonemia
54
Most common enzyme deficiency in urea cycle
Onithine transcarbomylase
55
Most severe enzyme deficiency in urea cycle
Carbamoyl phosphate synthetase 1 deficiency
56
Rx for hereditary hyperammonemia
Phenylbutyrate
| It gets converted to phenylacetate, binds to glutamine, and then excreted as phenylglutamine
57
Dse of adults with compromised liver function
| Due to cirrhosis, portal blood is shunted directly to systemic curculation
Acquired hyperammonemia
58
In the second phase of AA catabolism, ketogenic AA may be converted to _____, while glucogenic AA are converted to ____
Acetyl CoA or acetoacetyl CoA
| Pyruvate or intermediates of TCA
59
Purely ketogenic AA
Lysine
| Leucine
60
Glucogenic and ketogenic AA
```
Phenylalanine
Tyrosine
Isoleucine
Tryptophan
(Threonine in some textbooks)
```
61
Identify amino acid with these specialized products
| Creatibe, polyamines, nitric oxide
Arginine
62
Identify amino acid with these specialized products
| Coenzyme A, taurine, glutathione
Cysteine
63
Identify amino acid with these specialized products
| GABA, glutathione
Glutamate
64
Identify amino acid with these specialized products
| Heme, purines, creatine, glutathione, conjugated to bile acids, drugs, and other metabolites
Glycine
65
Identify amino acid with these specialized products
| Histamine
Histidine
66
Identify amino acid with these specialized products
| Carnitine
Lysine
67
Identify amino acid with these specialized products
| S-adenosylmethionine, creatine, polyamines
Methionine
68
Identify amino acid with these specialized products
| Sphingosine, purines, thymine
Serine
69
Identify amino acid with these specialized products
| Serotonin, niacin, melatonin
Tryptophan
70
Identify amino acid with these specialized products
| Catecholamines
Tyrosine
71
Identify amino acid with these specialized products
| Thyroid hormones
Tyrosine
72
Identify amino acid with these specialized products
| Melanin
Tyrosine
73
Identify amino acid with these specialized products
| Serotonin
Tryptophan
74
Identify amino acid with these specialized products
| Niacin
Tryptophan
75
Identify amino acid with these specialized products
| Melatonin
Typtophan
76
Identify amino acid with these specialized products
| Sphingosine
Serine
77
Identify amino acid with these specialized products
| Thymine
Serine
78
Identify amino acid with these specialized products
| SAM
Methionine
79
Catecholamine synthesis
| Tyrosine—>
3,4 dihydroxyphenylalanine (DOPA)
80
Catecholamine synthesis
Identify enzyme and co factor
Tyrosine—>DOPA
Tyrosine hydroxylase
| Tetrahydrobiopterin (THB)
81
Catecholamine synthesis
Identify enzyme and cofactor
3,4 DOPA—> Dopamine
DOPA decarboxylase
| Vitamin B6
82
Catecholamine synthesis
Identify enzyme and cofactor
Dopamine—> Norepinephrine
Dopamine beta hydroxylase
| Vitamin C/copper
83
Catecholamine synthesis
Identify enzyme and cofactor
Norepinephrine—>epinephrine
Phenylethanolamine N methyltransferase
SAM—>SAH
84
Catecholamine synthesis
Identify product
Dopamine —>
Norepinephrine
85
Catecholamine synthesis
Identify product
Norepinephrine—>
Epinephrine
86
Catecholamine degradation
| Catecholamines are inactivated by these 2 enzymes
```
Monoamine oxidase (MAO)
Catechol-O-methyltransferase(COMT)
```
87
Catecholamine degradation
| Norepinephrine and epinephrine are degraded to _______
VMA (Vanillylmandelic acid (VMA)
| Increased in pheochromocytoma
88
Catecholamine degradation
| Dopamine is degrade into ______
Homovanillic acid (HVA)
89
Amino acid disorders
| Enzyme deficiency in PKU
Phenylalanine hydroxylase
| Or tetrahydrobiopterin
90
Amino acid disorders
| Tyrosine becomes essential and phenylalanine accumulates
PKU
91
```
Amino acid disorder
Severe mental retardation
Failure to walk or talk
Seizures
Fair skin
Eczema
Musty body odor
```
PKU
92
Amino acid disorders
| Accumulation of this phenylketone in PKU gives the characteristic odor to urine
Phenylpyruvate
93
Amino acid disorders
| Dietary control for PKU aims to decrease _____ and increase ____
Phenylalanine
| Tyrosine
94
Amino acid disorders
| Congenital deficiency of this enzyme leads to alkaptonuria
Homogentisate oxidase
95
Amino acid disorders
| In alkaptonuria, there is acumulation of this substance
Homogentisic acid
96
Amino acid disorder
Dark urine on standing
Dark connective tissue (ochronosis)
Benign dse but may have debilitating arthalgia
Alkaptonuria
97
Amino acid disorders
| Tx for alkaptonuria
Reduce phenylalanine and tyrosine in diet
| Vit C for older children and adults
98
Dietary mgt for PKU
Low protein low carb
99
Identify disorder
Defective melanin synthesis from tyrosine, which may be from
Defective tyrosine transporters or
Absence of copper-requiring enzyme tyrosine
Albinism
100
Enzyme deficiency in albinism
Tyrosinase
101
Absence of pigment from hair, eyes, skin
| Increased risk for skin cancer
Albinism
102
Defect in methionine degradation keading to high plasma and urinary levels of homocysteine and methionine and low levels of cysteine
Homocystinuria
103
Homocystinuria leads to increased plama and urinary levels of _____ and ______ and low levels of ______
Homocysteine, methionine
| Cysteine
104
Either of these 2 enzymes deficiency leads to homocysteinuria
Methionine synthase
| Cystathionine B Synthase
105
Coenzyme of methionine synthase
Methylcobalamin
106
Coenzyme of cytathionine B synthase
Pyridoxal phosphate
107
Most common deficiency in homocysteinuria
Cystathionine B synthase
108
```
Ectopia lentis (downward displacement)
Faulty bone development and osteoporosis
Mental retardation
Tendency to form thrombi
Myocardial infarction
Stroke in children and young adults
```
Homocystinuria
109
Vitamin supplementation for homocystinuria
Vitamin B6, B12, folate
110
Diet in px with homocystinuria:
Low in _____
High in _______
Low in eggs, fish, meat
| High in plat based protein : soya, nuts
111
Cystine kidney stones —> staghorn calculi
Inherited defect of renal tubular amino acid transporter for Cystine, Ornithine, Lysine and Arginine in the PCT of kidneys
Cystinuria
112
Rx for cystinuria
Acetazolamide (to alkalinized the urine)
113
Enzyme deficiency that leads to a defect in the cinversion of methylmalonyl CoA to succinyl CoA
Methylmalonyl CoA mutase
114
Seizure, encephalopathy, stroke at age of 1 month to 1 year
Hypotonia, lethargy, failure to thrive, hepatosplenomegaly, monilial infections
Impaired metabolism of isoleucine, valine, threonine, methionine and odd chain FA
Methylmalonic acidemia
115
Tx for methylmalonic acidemia includes protein restriction of _____ g/kg/d with supplementation of ___ and ____^
0.5-1.5
| L carnitine, cobalamin
116
Enzyme deficiency in MSUD
a ketoacid dehydrogenase complex
117
Branched chain amino acids whose degradation are blocked in MSUD
Leucine, isoleucine, valine
118
Cyclic compounds formed by linkage of four pyrrole rings through methyne (-HC) bridges
Porphyrins
119
Enzymes catalase, peroxidase, guanylate cyclase all utilize this protein for their synthesis
Heme
120
Rate-limiting step in heme synthesis
Glycine + succinyl CoA —> D aminolevulinic acid
| Enzyme ALA synthase
121
Rate-limiting enzyme for heme synthesis and co factor
ALA Synthase
| Co factor: Pyridoxine
122
In heme synthesis, condensation of 2 molecules of D ALA leads to _______ by enzyme ____^^
Porphobilinogen
| Emzyme: ALA Dehydratase
123
1. Zinc-containing enzyme for the formation of porphobilinogen during heme synthesis
2. Its inhibitor
1. ALA dehydratase
| 2. Lead (heavy metal ions)
124
Ferrochelatase + ______ —> Heme
Protoporphyrin IX
125
Identify enzyme and product impaired in this defect of Heme synthesis
ALA dehydratase deficiency porphyria
ALA dehydratase
| Porphobilinogen
126
Identify enzyme and product impaired in this defect of Heme synthesis
Acute intermittent porphyria
PBG deaminase
127
Identify enzyme and product impaired in this defect of Heme synthesis
Congenital erythropoietic porphyria
Uropophyrinigen III cosynthase
| Uroporphyrinogen III
128
Identify enzyme and product impaired in this defect of Heme synthesis
Porphyria cutanea tarda
Uroporphyrinogen decarboxylase
| Coproporphyrinogen III
129
Identify enzyme and product impaired in this defect of Heme synthesis
Hereditary coproporphyria
Coproporphyrinogen oxidase
| Protoporphyrinogen IX
130
Identify enzyme and product impaired in this defect of Heme synthesis
Variegate porphyria
Protoporphyrinogen oxidase
| Protoporphyrin IX
131
Identify enzyme and product impaired in this defect of Heme synthesis
Erythropoietic protoporphyria
Ferrochelatase
| Heme
132
Subcellular site of heme synthesis
Cytosol: D ALA to coproporphyrinogen III
| the rest: mitchondria
133
2 enzymes in heme synthesis most sensitive to lead poisoning
ALA dehytrase and ferrochelatase
134
Increased urinary ALA and free erythrocyte porphyrins
Microcytic anemia with basophilic stippling of RBCs
Headached, memory loss
Peripheral neuropathy, claw hand, wrist drop
Nausea, abdominal pain, diarrhea
Deposits in gums, epiphyses
Lead poisoning
135
Most common porphyria
Porphyria cutanea tarda
136
Cutaneous fragility
| Blistering of hands, forearms, face
Porohyria cutanea tarda
137
Enzyme deficiency in porphyria cutanea tarda
Uroporphyrinogen decarboxylase
138
Accumulation of PBG and D ALA in the urine
Neuropsychiatric symptoms
Urine darkens when exposed to light
Acute intermittent porphyria
139
Enzyme deficiency in acute intermittent porphyria
Hydroxymethylbilane synthase (uroporphyrinogen I synthase)
140
Tx for mild and severe attack of acute intermittent porphyria
Mild: high dose glucose
Severe: hematin
141
After 120 days, RBCs are taken up and degraded by the RES, particularly in these 2 organs
Liver spleen
142
Heme degradation releases this by-product
Carbon monoxide
143
Bilirubin is transported in the blood by binding to _____
Albumin
144
In the liver, bilirubin binds to intracellular proteins, particularly to ___
Ligandin
145
Enzyme for the formation of bilirubin diglucuronide
Bilirubin glucuronyltransferase
146
Step in heme degradation that is most susceptible to liver disease
Secretion of bilirubin into bile
147
In the gut, bilirubin —> _____ (colorless)
Urobilinogen
148
Intestinal bacteria oxidize urobilinogen—> ______ (brown)
Stercolin
149
Some urobilinogen is reabsorbed from the blood and enters the ________
Portal circulation
150
Remaining urobilinogen is transported by the blood to the kidney, where it is converted to _______ (yellow)
Urobilin
151
Enzyme deficient in crigler najjar, gilbert syndrome
Bilirubin glucuronyltransferase
152
_________ reaction is used to measure bilirubin in serum
Van den Bergh Reaction
153
In Van den Bergh reaction,
Assay with no methanol measures ____
Assay with methanol measures ______
Difference in two measures _______
Direct bilirubin
Total bilirubin
Indirect bilirubin
154
Defect in bilirubin uptake/conjugation/secretion
| Dubin Johnson Syndrome
Secretion
155
Defect in bilirubin uptake/conjugation/secretion
| Neonatal jaundice
Conjugation
156
Defect in bilirubin uptake/conjugation/secretion
| Crigler Najjar
Conjugation
157
Defect in bilirubin uptake/conjugation/secretion
| Gilbert syndrome
Conjugation
158
Direct/indirect hyperbilirubinemia
| Biliary tree obstruction
Direct
159
Direct/indirect hyperbilirubinemia
| Dubin Johnson Syndrome
Direct
160
Direct/indirect hyperbilirubinemia
| Rotor syndrome
Direct
161
Direct/indirect hyperbilirubinemia
| Hemolytic anemia
Indirect
162
Direct/indirect hyperbilirubinemia
| Neonatal physiologic jaundice
Indirect
163
Direct/indirect hyperbilirubinemia
| Crigler Najjar
Indirect
164
Direct/indirect hyperbilirubinemia
| Gilbert syndrome
Indirect
165
Direct/indirect hyperbilirubinemia
| Toxic hyperbilirubinemia
Indirect
166
Insulin/Glucagon
| Source is Beta cells of islets
Insulin
167
Insulin/Glucagon
| Source is alpha cells of islets
Glucagon
168
Insulin/Glucagon
| Two polypeptide chains with 51 AA linked together by 2 disulfide bridges
Insulin
169
Insulin/Glucagon
| Single polypeptide chain with 29 AA
Glucagon
170
Insulin/Glucagon
| Second messenger is cAMP
Glucagon
171
Insulin/Glucagon
| 2nd messenger is tyrosine kinase
Insulin
172
Insulin/Glucagon
| Stimulated by cholecystokinin and gastric inhibitory peptide
Insulin
173
Insulin/Glucagon
| Stimulated by hypoglycemia
Glucagon
174
```
Insulin/Glucagon
Increases
Gluconeogenesis
Glycogenolysis
Uptake of AA
Beta oxidation
Ketigenesis
```
Glucagon
175
```
Insulin/Glucagon
Increases
Glucose uptake
Glycigenesis
Protein synthesis
Lipogenesis
```
Insulin
176
Insulin/Glucagon
Inhibits gluconeogenesis
Glycogenolysis
Beta oxidation
Insulin
177
Insulin/Glucagon
| Inhibits glycogenesis
Glucagon
178
3 hormones with similar effects as glucagon
Epinephrine
| Cortisol GH
179
During fed state, the brain uses _____ as exclusive source of fuel
Glucose
180
During fasting, the brain uses_____ and _____ as sources of fuel
Glucose, ketone
181
TAG stores and FA are used by the brain for fuel
| T/F
F. They cant cross BBB
182
During fed state, the PPP is activated in the liver and adipose to provide ______
NADPH
183
Majore source of energy in adipose tissue during marathon
FFA
184
Principal site of metabolism of BCAA
Skeletal muscle
185
Identify disorder
Defective melanin synthesis from tyrosine, which may be from
Defective tyrosine transporters or
Absence of copper-requiring enzyme tyrosine
Albinism
186
Enzyme deficiency in albinism
Tyrosinase
187
Absence of pigment from hair, eyes, skin
| Increased risk for skin cancer
Albinism
188
Defect in methionine degradation keading to high plasma and urinary levels of homocysteine and methionine and low levels of cysteine
Homocystinuria
189
Homocystinuria leads to increased plama and urinary levels of _____ and ______ and low levels of ______
Homocysteine, methionine
| Cysteine
190
Either of these 2 enzymes deficiency leads to homocysteinuria
Methionine synthase
| Cystathionine B Synthase
191
Coenzyme of methionine synthase
Methylcobalamin
192
Coenzyme of cytathionine B synthase
Pyridoxal phosphate
193
Most common deficiency in homocysteinuria
Cystathionine B synthase
194
```
Ectopia lentis (downward displacement)
Faulty bone development and osteoporosis
Mental retardation
Tendency to form thrombi
Myocardial infarction
Stroke in children and young adults
```
Homocystinuria
195
Vitamin supplementation for homocystinuria
Vitamin B6, B12, folate
196
Diet in px with homocystinuria:
Low in _____
High in _______
Low in eggs, fish, meat
| High in plat based protein : soya, nuts
197
Cystine kidney stones —> staghorn calculi
Inherited defect of renal tubular amino acid transporter for Cystine, Ornithine, Lysine and Arginine in the PCT of kidneys
Cystinuria
198
Rx for cystinuria
Acetazolamide (to alkalinized the urine)
199
Enzyme deficiency that leads to a defect in the cinversion of methylmalonyl CoA to succinyl CoA
Methylmalonyl CoA mutase
200
Seizure, encephalopathy, stroke at age of 1 month to 1 year
Hypotonia, lethargy, failure to thrive, hepatosplenomegaly, monilial infections
Impaired metabolism of isoleucine, valine, threonine, methionine and odd chain FA
Methylmalonic acidemia
201
Tx for methylmalonic acidemia includes protein restriction of _____ g/kg/d with supplementation of ___ and ____^
0.5-1.5
| L carnitine, cobalamin
202
Enzyme deficiency in MSUD
a ketoacid dehydrogenase complex
203
Branched chain amino acids whose degradation are blocked in MSUD
Leucine, isoleucine, valine
204
Cyclic compounds formed by linkage of four pyrrole rings through methyne (-HC) bridges
Porphyrins
205
Enzymes catalase, peroxidase, guanylate cyclase all utilize this protein for their synthesis
Heme
206
Rate-limiting step in heme synthesis
Glycine + succinyl CoA —> D aminolevulinic acid
| Enzyme ALA synthase
207
Rate-limiting enzyme for heme synthesis and co factor
ALA Synthase
| Co factor: Pyridoxine
208
In heme synthesis, condensation of 2 molecules of D ALA leads to _______ by enzyme ____^^
Porphobilinogen
| Emzyme: ALA Dehydratase
209
1. Zinc-containing enzyme for the formation of porphobilinogen during heme synthesis
2. Its inhibitor
1. ALA dehydratase
| 2. Lead (heavy metal ions)
210
Ferrochelatase + ______ —> Heme
Protoporphyrin IX
211
Identify enzyme and product impaired in this defect of Heme synthesis
ALA dehydratase deficiency porphyria
ALA dehydratase
| Porphobilinogen
212
Identify enzyme and product impaired in this defect of Heme synthesis
Acute intermittent porphyria
PBG deaminase
213
Identify enzyme and product impaired in this defect of Heme synthesis
Congenital erythropoietic porphyria
Uropophyrinigen III cosynthase
| Uroporphyrinogen III
214
Identify enzyme and product impaired in this defect of Heme synthesis
Porphyria cutanea tarda
Uroporphyrinogen decarboxylase
| Coproporphyrinogen III
215
Identify enzyme and product impaired in this defect of Heme synthesis
Hereditary coproporphyria
Coproporphyrinogen oxidase
| Protoporphyrinogen IX
216
Identify enzyme and product impaired in this defect of Heme synthesis
Variegate porphyria
Protoporphyrinogen oxidase
| Protoporphyrin IX
217
Identify enzyme and product impaired in this defect of Heme synthesis
Erythropoietic protoporphyria
Ferrochelatase
| Heme
218
Subcellular site of heme synthesis
Cytosol: D ALA to coproporphyrinogen III
| the rest: mitchondria
219
2 enzymes in heme synthesis most sensitive to lead poisoning
ALA dehytrase and ferrochelatase
220
Increased urinary ALA and free erythrocyte porphyrins
Microcytic anemia with basophilic stippling of RBCs
Headached, memory loss
Peripheral neuropathy, claw hand, wrist drop
Nausea, abdominal pain, diarrhea
Deposits in gums, epiphyses
Lead poisoning
221
Most common porphyria
Porphyria cutanea tarda
222
Cutaneous fragility
| Blistering of hands, forearms, face
Porohyria cutanea tarda
223
Enzyme deficiency in porphyria cutanea tarda
Uroporphyrinogen decarboxylase
224
Accumulation of PBG and D ALA in the urine
Neuropsychiatric symptoms
Urine darkens when exposed to light
Acute intermittent porphyria
225
Enzyme deficiency in acute intermittent porphyria
Hydroxymethylbilane synthase (uroporphyrinogen I synthase)
226
Tx for mild and severe attack of acute intermittent porphyria
Mild: high dose glucose
Severe: hematin
227
After 120 days, RBCs are taken up and degraded by the RES, particularly in these 2 organs
Liver spleen
228
Heme degradation releases this by-product
Carbon monoxide
229
Bilirubin is transported in the blood by binding to _____
Albumin
230
In the liver, bilirubin binds to intracellular proteins, particularly to ___
Ligandin
231
Enzyme for the formation of bilirubin diglucuronide
Bilirubin glucuronyltransferase
232
Step in heme degradation that is most susceptible to liver disease
Secretion of bilirubin into bile
233
In the gut, bilirubin —> _____ (colorless)
Urobilinogen
234
Intestinal bacteria oxidize urobilinogen—> ______ (brown)
Stercolin
235
Some urobilinogen is reabsorbed from the blood and enters the ________
Portal circulation
236
Remaining urobilinogen is transported by the blood to the kidney, where it is converted to _______ (yellow)
Urobilin
237
Enzyme deficient in crigler najjar, gilbert syndrome
Bilirubin glucuronyltransferase
238
_________ reaction is used to measure bilirubin in serum
Van den Bergh Reaction
239
In Van den Bergh reaction,
Assay with no methanol measures ____
Assay with methanol measures ______
Difference in two measures _______
Direct bilirubin
Total bilirubin
Indirect bilirubin
240
Defect in bilirubin uptake/conjugation/secretion
| Dubin Johnson Syndrome
Secretion
241
Defect in bilirubin uptake/conjugation/secretion
| Neonatal jaundice
Conjugation
242
Defect in bilirubin uptake/conjugation/secretion
| Crigler Najjar
Conjugation
243
Defect in bilirubin uptake/conjugation/secretion
| Gilbert syndrome
Conjugation
244
Direct/indirect hyperbilirubinemia
| Biliary tree obstruction
Direct
245
Direct/indirect hyperbilirubinemia
| Dubin Johnson Syndrome
Direct
246
Direct/indirect hyperbilirubinemia
| Rotor syndrome
Direct
247
Direct/indirect hyperbilirubinemia
| Hemolytic anemia
Indirect
248
Direct/indirect hyperbilirubinemia
| Neonatal physiologic jaundice
Indirect
249
Direct/indirect hyperbilirubinemia
| Crigler Najjar
Indirect
250
Direct/indirect hyperbilirubinemia
| Gilbert syndrome
Indirect
251
Direct/indirect hyperbilirubinemia
| Toxic hyperbilirubinemia
Indirect
252
Insulin/Glucagon
| Source is Beta cells of islets
Insulin
253
Insulin/Glucagon
| Source is alpha cells of islets
Glucagon
254
Insulin/Glucagon
| Two polypeptide chains with 51 AA linked together by 2 disulfide bridges
Insulin
255
Insulin/Glucagon
| Single polypeptide chain with 29 AA
Glucagon
256
Insulin/Glucagon
| Second messenger is cAMP
Glucagon
257
Insulin/Glucagon
| 2nd messenger is tyrosine kinase
Insulin
258
Insulin/Glucagon
| Stimulated by cholecystokinin and gastric inhibitory peptide
Insulin
259
Insulin/Glucagon
| Stimulated by hypoglycemia
Glucagon
260
```
Insulin/Glucagon
Increases
Gluconeogenesis
Glycogenolysis
Uptake of AA
Beta oxidation
Ketigenesis
```
Glucagon
261
```
Insulin/Glucagon
Increases
Glucose uptake
Glycigenesis
Protein synthesis
Lipogenesis
```
Insulin
262
Insulin/Glucagon
Inhibits gluconeogenesis
Glycogenolysis
Beta oxidation
Insulin
263
Insulin/Glucagon
| Inhibits glycogenesis
Glucagon
264
3 hormones with similar effects as glucagon
Epinephrine
| Cortisol GH
265
During fed state, the brain uses _____ as exclusive source of fuel
Glucose
266
During fasting, the brain uses_____ and _____ as sources of fuel
Glucose, ketone
267
TAG stores and FA are used by the brain for fuel
| T/F
F. They cant cross BBB
268
During fed state, the PPP is activated in the liver and adipose to provide ______
NADPH
269
Majore source of energy in adipose tissue during marathon
FFA
270
Principal site of metabolism of BCAA
Skeletal muscle
