Metabolism Review Flashcards
1
Q
What cellular processes happen in both mitochondria and cytoplasm
A
Urea cycle, heme synthesis, gluconeogenesis
2
Q
What processes happen in only mitochondria
A
Beta oxidation of fatty acids, acetyl coa production, krebs cycle, oxidative phosphorylation.
3
Q
Carboxylase enzyme cofactor
A
Add CO2 to things – require biotin
4
Q
Rate determining enzyme of glycolysis
A
PFK-1, activated by F26bp, amp, inhibited by atp, citrate
5
Q
Rate determining enzyme of gluconeogenesis
A
F16bisphosphatase, activated by ATP and acetyl coa. Inhibited by AMP and fructose 2,6 bisphosphate
6
Q
Rate determining enzyme of TCA
A
Isocitrate dehydrogenase, activated by ADP
7
Q
Rate determining enzyme of glycogenesis
A
Glycogen synthase
8
Q
Rate determining enzyme of glycogenolysis
A
Glyogen phosphorylase
9
Q
Rate determining enzyme of HMP shunt
A
Glucose 6 phosphate dehydrogenase
10
Q
Rate determinign enzyme of de novo purine synthesis
A
PRPP amidotransferase
11
Q
Urea cycle rate limiting enzyme
A
Carbamoyl phosphate synthetase
12
Q
Fatty acid synthesis rate determining enzyme
A
Acetyl coa carbodylase
13
Q
Fatty acid oxidation rate determining enzyme
A
Carnitine acyltranferase
14
Q
Fatty acid oxidation rate determining enzyme
A
Carnitine acyltranferase
15
Q
How much atp does aerobic metabolism of glucose produce?
A
32 via malate-aspartate shuttle (heart and liver), 30 via G3P shuttle (muscle
16
Q
How much atp does anaerobic glycolysis produce
A
Costs 2, makes 4, net 2.
17
Q
How does arsenic affect metabolism
A
Arsenic causes glycolysis to net zero ATP and also inhibits lipoic acid
18
Q
What do each of these active carriers produce? ATP NADH CoA, lipoamide Biotin THF SAM TPP
A
Phosphate electrons acyl group (b7) CO2 1 carbon units CH3 groups Aldehydes (b1)
19
Q
NAD vs NADPH
A
NAD (from b3) ususally used in catabolic processes to carry away electrons as NADH
NADPH is usually used in anabolic processes (steroid and fatty acid synthesis), the respiratory burst, cyp450, and glutathione reductase
20
Q
Hexokinase vs glucokinase
A
Hexokinase has low Km (high affinity) and low total velocity. In muscle so it can use glucose as it exists in the body. Not induced by insulin, inhibited by G6P.
Glucokinase has high Km (low affinity) and high velocity. Only in liver. Induced by insulin after a meal to store glucose in cells for use as glycogen. Not inhibited by g6P.
21
Q
MODY
A
Maturity onset diabetes of the young – defect in glucokinase so not enough glucose taken into liver. Hyperglycemia.
22
Q
MODY
A
Maturity onset diabetes of the young – defect in glucokinase so not enough glucose taken into liver. Hyperglycemia.
23
Q
Draw glycolysis out
A
Pay special attention to the steps that require ATP (G, G3P; F6P, F16BP)
and make atp (13BPG, 3PG; PEP,pyr)
24
Q
Cofactors required in pyruvate dehydrogenase complex
A
TPP (B1) FAD (B2) NAD (B3) CoA (B5) Lipoic Acid (inhibted by arsenic, causes vomiting, rice water stools, garlic breath).
25
Where is pyruvate dehydrogenase located?
| How is pyruvate dehydrogenase activated? What is the product made?
Located in the mitochondrial membrane
By a exercise which causes high NAD/NADH ratio, increased ADP, increased Ca.
Acetyl CoA.
26
Pyruvate dehydrogenase deficiency findings and treatment
Causes lactic acidosis (via LDH) and build up of alanine (via ALT).
Neurologic symptoms too.
Treat by supplementing ketogenic amino acids (lysine and leucine) or ketogenic foods (high fat)
27
Pyruvate dehydrogenase deficiency findings and treatment
Causes lactic acidosis (via LDH) and build up of alanine (via ALT).
Neurologic symptoms too.
Treat by supplementing ketogenic amino acids (lysine and leucine) or ketogenic foods (high fat), which bypass PDH complex and go directly into TCA
28
Products of pyruvate metabolism
Lactate via LDH (anaerobic metabolism in retina, lens, RBC, renal medulla)--requires B3
Alanine via ALT (used to take amino groups from muscle to liver)-- requires B6
OAA via Pyruvate carboxylase (to be used in gluconeogenesis) -- requires biotin
Acetyl-CoA via Pyruvate dehydrogenase
29
TCA Products
3 NADH, 1 FADH2, 2 CO2, 1GTP per acetyl coa, 2x for 1 glucose.
30
Electron transport chain.
Electrons enter intermembrane space through complexes 1-4 from the inner mitochondrial membrane. Complex 1 is where NADH goes, complex 2 (succinate dehydrogenase) is where FADH goes. Complex 4 passes electrons to O2 to make H2O. Generates H gradient in intermembrane space which pass through ATP synthase and make ATP.
31
Electron transport chain.
Electrons enter intermembrane space through complexes 1-4 from the inner mitochondrial membrane. Complex 1 is where NADH goes, complex 2 (succinate dehydrogenase) is where FADH goes. Complex 4 passes electrons to O2 to make H2O. Generates H gradient in intermembrane space which pass through ATP synthase and make ATP.
32
ETC complex inhibitors
Rotenone inhibits complex 1
Antimycin inhibits complex 3
Cyanide/CO inhibit complex 4
Oligomycin inhibits ATPsynthase
33
Uncoupling agents
Aspirin overdose
Thermogenin
2,4 Dinitrophenol
increase O2 consumption because they decrease proton gradient, but No ATP generated. Energy dissipated as heat
34
Gluconeogenesis key enzymes
Pyruvate carboxylase (in mitochondria), requires biotin
PEPcarboxykinase in cytosol (requires GTP)
Fructose 1,6 BPase
Glucose 6 Phosphatase (not found in muscle)
35
Why can't gluconeogenesis happen in muscle?
Muscle lacks glucose 6 phosphatase.
36
How do even chain fatty acids contribute to gluconeogenesis
They don't. They can only yield acetyl coa equivalents.
37
How do odd chain fatty acids contribute to gluconeogenesis
When broken down, yield proprionyl coa, which can enter TCA as succinyl CoA, regenerate OAA.
38
How much ATP does 1 NADH produce?
| FADH?
2. 5
| 1. 5
39
HMP Shunt
Provides a source of NADPH when glucose is abundant to serve in fatty acid and steroid synthesis, glutathione reduction in RBC. Also used to generate ribose for nucleotide synthesis.
40
HMP Shunt
Provides a source of NADPH when glucose is abundant to serve in fatty acid and steroid synthesis, glutathione reduction in RBC. Also used to generate ribose for nucleotide synthesis.
41
Oxidative phase of HMP shunt
Irreversible phase, G6P turned into Ribulose 5 P, generating 2 NADPH, via Glucose 6 Phosphate Dehydrogenase.
42
Nonoxidative phase of HMP shunt
Revesible phase, ribulose 5 phosphate turned into ribose 5 phosphate by phosphopentose isomerase. G3P and F6P generated for glycolysis by transketolases.
43
Nonoxidative phase of HMP shunt
Revesible phase, ribulose 5 phosphate turned into ribose 5 phosphate by phosphopentose isomerase. G3P and F6P generated for glycolysis by transketolases.
44
Components of respiratory burst
O2 turned into O2- by NADPH oxidase, O2-turned into H2O2 by SOD, H2O2 turned into HOCl by MPO.
45
How is H2O2 neutralized?
By Glutathione peroxidase.
46
G6PD deficiency
The most common enzyme deficiency. X-linked recessive disorder. Causes Hb to be oxidized and build up in cells as heinz bodies. Bite cells from spleen especially after sulfa drugs, malaria drugs, fava beans. Hemolytic anemia.
47
Fructokinase deficiency
Causes fructose to build up in blood and urine (can't be P'd to F1P and stay in cells). Totally benign.
48
Aldolase B deficiency
More serious (though less serious than galactose metabolism problems). F1P accumulates in cells, decrease in available phosphate, which blocks glycogenolysis and gluconeogenesis.
Hypoglycemia, jaundice, cirrhosis, vomiting, especially after fruit juices and honey.
URINE DIPSTICK NEGATIVE (only tests for glucose).
Treat by decreasing fructose and sucrose.
49
Essential Fructosuria
Fructokinase deficiency Causes fructose to build up in blood and urine (can't be P'd to F1P and stay in cells). Totally benign.
50
Fructose intolerance
Aldolase B deficiency. More serious (though less serious than galactose metabolism problems). F1P accumulates in cells, decrease in available phosphate, which blocks glycogenolysis and gluconeogenesis.
Hypoglycemia, jaundice, cirrhosis, vomiting, especially after fruit juices and honey.
URINE DIPSTICK NEGATIVE (only tests for glucose).
Treat by decreasing fructose and sucrose.
51
Galactokinase deficiency
Galactose cant be turned into Galactose 1 P, so galACTitol builds up. Relatively mild.
Symptoms are: lack of a social smile, galactose in blood/urine, infantile catarACTs.
52
Classic galactosemia
Absence of galactose 1 phosphate uridyltransferase. Damage is caused by accumulation of galactitol.
Causes failure to thrive, jaundice, hepatomegaly, infantile catarACTs.
Treat by exclusing galactose and lactose from diet.
53
Classic galactosemia
Absence of galactose 1 phosphate uridyltransferase. Damage is caused by accumulation of galactitol.
Causes failure to thrive, jaundice, hepatomegaly, infantile catarACTs.
Treat by exclusing galactose and lactose from diet.
54
How to create sorbitol?
What is it used for?
Any problems with sorbitol
From glucose using aldose reductase. Its an alternative method of trapping glucose in cells if it is high. Many tissues have sorbitol dehydrogenase to convert it to fructose, however, schwann cells, lens, and retina don't have it. Causes osmotic damage. This is what happens in diabetes.
55
How to create sorbitol?
What is it used for?
Any problems with sorbitol
From glucose using aldose reductase. Its an alternative method of trapping glucose in cells if it is high. Many tissues have sorbitol dehydrogenase to convert it to fructose, however, schwann cells, lens, and retina don't have it. Causes osmotic damage. This is what happens in diabetes.
56
Glucogenic Essential Amino Acids
Valine, Histidine, Methionine
57
Glucogenic/Ketogenic Essential Amino Acids
Isoleucine, tryptophan, threonine, phenylalanine.
58
Ketogenic Essential Amino Acids
Lysine/leucine
59
Acidic Amino Acids
Glutamate, Aspartate
Negatively charged at physiologic pH
60
Basic Amino Acids
Histidine ** , lysine, arginine.
Arginine and lysine are in histones because they bind negatively charged DNA.
Postively charged at physiologic pH
61
Urea cycle purpose
to turn amino groups into urea for excretion. Takes place in liver, both in mitochondria and cytoplasm.
62
Urea cycle purpose
to turn amino groups into urea for excretion. Takes place in liver, both in mitochondria and cytoplasm.
63
Draw the urea cycle
Ordinarily, careless (created by NH3 and CO2) crappers (are) also (frivolous) about (urination)
64
Draw the urea cycle
REQUIRES N-Acetyl glutamate to turn NH3 into carbamoyl phosphate!!!!!!!! Ordinarily, careless (created by NH3 and CO2) crappers (are) also (frivolous) about (urination)
65
Transport by ammonia by alanine and glutamate.
Draw it.
66
Hyperammonemia- cause/sxs/ treatment
Can be acquired (liver disease) or genetic (enzyme deficiency). Results in excess NH4 which depletes alpha ketoglutarate and inhibits TCA cycle.
Causes tremor, slurring of speech, somnolence, vomiting, cerebral edema.
Treat by limiting protein in diet, or by lactulose to acidify GI tract and trap NH4 for excretion.
Also treat with benzoate or phenyl butyrate (bind AA for excretion).
67
N-Acetylglutamate deficiency
Required cofactor for carbamoyl phosphate synthetase I. Deficiency leads to hyperammonemia. Presentation is identical to CPS I deficiency but this has increased ornithine with normal urea cycle enzymes.
68
Ornithine transcarbamylase deficiency
Most common urea cycle disorder. X linked recessive. Interferes with body's ability to excrete ammonia. Often evident in first few days of life. Excess carbamoyl phosphate is converted to orotic acid which causes increased orotic acid in blood and urine, decreased BUN, symptoms of hyperammonemia. NO MEGALOBLASTIC ANEMIA (seen in orotic aciduria).
69
Ornithine transcarbamylase deficiency
Most common urea cycle disorder. X linked recessive. Interferes with body's ability to excrete ammonia. Often evident in first few days of life. Excess carbamoyl phosphate is converted to orotic acid which causes increased orotic acid in blood and urine, decreased BUN, symptoms of hyperammonemia. NO MEGALOBLASTIC ANEMIA (seen in orotic aciduria).
70
Ornithine transcarbamylase deficiency
Most common urea cycle disorder. X linked recessive. Interferes with body's ability to excrete ammonia. Often evident in first few days of life. Excess carbamoyl phosphate is converted to orotic acid which causes increased orotic acid in blood and urine, decreased BUN, symptoms of hyperammonemia. NO MEGALOBLASTIC ANEMIA (seen in orotic aciduria).
71
Phenylalanine metabolism
Becomes tyrosine, DOPA, Dopamine, Norepinephrine, epinephrine.
72
Tryptophan becomes
Niacin (B6 cofactor)
| Serotonin (BH4, B6 cofactors)
73
Histidine becomes
Histamine (with B6 cofactor)
74
Glycine becomes
Porphyrin (with B6), then heme
75
Glutamate becomes
GABA (with b6)
| Glutathione
76
Arginine becomes
urea, creatinine, nitric oxide (with BH4)
77
Vitamin B6 deficiency causes
Sideroblastic anemia, needed for ALAS function to create ALA from succinyl CoA.
78
Catecholamine synthesis
Draw it.
79
Phenylketonuria
Deficiency in phenylalanine hydroxylase or a decrease in BH4 cofactor (malignant PKU), build up of phenylalanine leads to increased synthesis of phenylketones. Tyrosine becomes an essential amino acid.
Findings: intellectual disability, musty body odor, seizures, fair skin, eczema.
Treat by decreasing phe, increasing tyr in diet.
80
Maternal PKU
Lack of proper dietary therapy during pregnancy. Causes microcephaly, intellectual disability, growth retardation
81
Alkaptonuria (ochonosis)
Deficiency of homogentisate oxidase in the tyrosine degradation pathway. Build up of homogentisic acid
82
Alkaptonuria (ochonosis)
benign Deficiency of homogentisate oxidase in the tyrosine degradation pathway. Build up of homogentisic acid. Causes dark connective tissue, brown sclerae, urine turns black.
May have debilitating arthralgias (homogentisic acid is tocix to cartilage).
83
Homocystinuria
Caused by multiple things:
1) Cystathionine synthase deficiency (treat with decreasing methionine, increasing cysteine, increasing b12/folate.
2) mutation in cystathionine synthase which reduces affinity for pyridoxal phosphate (increase B6 and cysteine)
3) Homocysteine methyltransferase deficiency (treatment, increase methionine)
Causes intellectual disability, osteoporosis, tall stature, kyphosis, lens subluxation, MI
84
Cystinuria
Defect of renal PCT and intestinal aa transporter for COLA (cysteine, ornithine, lysine, arginine).
Excess cystine in urine can cause hexagonal cystine stones.
Urinary cyanide-nitroprusside test is diagnostic. Treat with urinary alkalinization.
85
Maple Syrup Urine Disease
Blocked degradation of branched amino acid (isoleucine, leucine, valine) due to alphaketoacid dehydrogenase. Severe CNS deficits, lethargy, seizures.
86
Maple Syrup Urine Disease
Blocked degradation of branched amino acid (isoleucine, leucine, valine) due to alphaketoacid dehydrogenase needs B1. Severe CNS deficits, lethargy, seizures.
Treat by supplementing B1 and avoiding BCAAs.
87
Phenylketonuria
Deficiency in phenylalanine hydroxylase or a decrease in BH4 cofactor (malignant PKU), build up of phenylalanine leads to increased synthesis of phenylketones. Tyrosine becomes an essential amino acid.
Findings: intellectual disability, musty body odor, seizures, fair skin, eczema
Also have growth retardation due to low thyroxine!
Treat by decreasing phe, increasing tyr in diet.
88
Maple Syrup Urine Disease
Blocked degradation of branched amino acid (isoleucine, leucine, valine) due to alphaketoacid dehydrogenase needs B1. Severe CNS deficits, lethargy, seizures.
Treat by supplementing B1 and avoiding BCAAs.
89
Do fat-soluble vitamins have higher or lower toxicity than water soluble vitamins?
Higher toxicity.
| Mineral oil intake can cause fat soluble vitamin deficiencies
90
What do B complex vitamin deficiencies usually cause?
Glossitis, dermatitis, diarrhea
91
Vitamin A function
Retinol. Constituent of visual pigments and essential for normal differentiation of epithelial cells.
92
Vitamin A deficiency
Night blindness, xerosis cutis, corneal degeneration (keratomalacia), immune suppression
93
Vitamin A excess
Teratogenic (cleft palate, hox gene interference). Scaly skin, osteoporosis, PSEUDOTUMOR CEREBRI
94
Vitamin B1
Thiamine. Used in TPP, a cofactor for severe dehydrogenase enzymes (pyruvate dyhydrogenase) AKG dehydrogenase, Transketolase (HMP shunt), and branched chain ketoacid dehydrogenase.
95
Vitamin B1 deficiency
Can happen in alcoholics and malnutrition. Causes impaired glucose breakdown (so give B1 to alcoholics before glucose). Highly aerobic tissues like brain and heart affected first. Causes beriberi and wenicke-korsakoff syndrome. Diagnosed with increased RBC transketolase activity after B1 administration.
96
Wernicke-Korsakoff Syndrome
B1 deficiency causes ataxia, opthamoplegia, confusion. Add confabulation and dementia/amnesia. Degeneration of mammillary bodies.
97
Wernicke-Korsakoff Syndrome
B1 deficiency causes ataxia, opthamoplegia, confusion. Add confabulation and dementia/amnesia. Degeneration of mammillary bodies.
98
Dry beriberi
Polyneuritis, symmetric muscle wasting
99
Wet beriberi
High output cardiac failure (dilated cardiomyopathy) and edema.
100
Wet beriberi
High output cardiac failure (dilated cardiomyopathy) and edema.
101
Vitamin B2
Riboflavin. Used in FAD and FMN in redox reactions (succinate dehydrogenase).
102
Vitamin B2 deficiency
Chielosis (inflammation of the lips and fissures at the corners), corneal vascularization.
103
Vitamin B2 deficiency
Cheilosis (inflammation of the lips and fissures at the corners), corneal vascularization.
104
Vitamin B2 deficiency
Cheilosis (inflammation of the lips and fissures at the corners), corneal vascularization.
105
Vitamin B3
Niacin. Constituent of NAD/NADP used in redox reactions. Derived from Tryptophan and synthesis requires B2 and B6. Lowers levels of VLDL and increases HDL
106
B3 Deficiency
Glossitis. Severe deficiency can be caused by Hartnup disease (decreased abs of tryptophan), carcinoid syndrome (tryptophan metabolism increased), and isoniazid (decreases B6).
Causes symptoms of pellagra: Diarrhea, dermatitis, and dementia.
107
B3 Excess
Facial flushing (induced by prostaglandin), hyperglycemia, hyperuricemia
108
Vitamin B5
Pantothenate. Essential component of CoA (for acyl tranfers), and fatty acid synthase.
109
Vitamin B5 deficiency
Dermatitis, enteritis, alopecia, adrenal insufficiency
110
Vitamin B6
Pyridoxine, converted to pyridoxal phosphate, a cofactor used in transamination (AST/ALT), decarboylation, glycogen phosphorylase. Used in the synthesis of cystathionine, heme, niacin, histamine, neurotransmitters.
111
Vitamin B6 deficiency
Convulsions, hyperirritability, peripheral neuropathy (especially with isoniazid), SIDEROBLASTIC ANEMIA DUE TO IMPAIRED HB SYNTHESIS.
112
Vitamin B6 deficiency
Convulsions, hyperirritability, peripheral neuropathy (especially with isoniazid), SIDEROBLASTIC ANEMIA DUE TO IMPAIRED HB SYNTHESIS.
113
Vitamin B7
Biotin. Cofactor for carboxylation which add one carbon.
114
Vitamin B7 Deficiency
Relatively rate, causes dermatitis, alopecia, enteritis. Caused by antibiotic use or excessive ingestion of egg whites.
115
Vitamin B9
Folic acid, converted to THF, a coenzyme for 1 carbon transfer/methylation reactions. Needed for synthesis of bases in DNA/RNA. Found in green leafy vegetables.
116
Vitamin B9 deficiency
Megaloblastic anemia, hypersegmented neutrophils, glossitis, no neurologic symtpoms. Increased homocysteine. Most common vitamin deficiency in the US.
Can be caused by several drugs (phenytoin, sulfonamides, methotrexate).
117
Vitamin B12
Cobalamin. Cofactor for homocysteine methyltransferase and methylmalonyl CoA mutase.
Found in animal products. Intrinsic factor for absorption in terminal ileum
118
Vitamin B12 deficiency
Megaloblastic anemia, hypersegmented neutrophils, paresthesias and subacute combined degenration (degeneration of dorsal columns, LCSTs, due to abnormal myelin). Increases homocysteine and methylmalonic acid.
Pernicious anemia, diphyllobothrium latum.
119
Vitamin C
Antioxidant, facilitates iron absorption (reduces it to Fe2+). Necessary for -OH of lysine and proline in collagen synthesis. Necessary for dopamine beta hydroxylase which converts dopamine to NE.
Can treat methemoglobinemia by reducing Fe3+ to Fe2+
120
Vitamin C deficiency
Scurvy. Swollen gums, bruising, hemarthroses, anemia, poor wound healing.
121
Vitamin C excess
Nausea, vomiting, diarrhea, calcium oxalate nephrolithiasis.
122
Vitamin D
D2 from plants
D3 from milk and skin (stratum basale)
Increases intestinal absorption of calcium and phosphate.
123
Vitamin D deficiency
Rickets/osteomalatia. Hypocalcemic tetany. Breastfed infants must receive vitamin D!
124
vitamin D excess
Hypercalcemia, loss of appetite. Seen in sarcoidosis because epitheloid histiocytes activate vitamin d.
125
vitamin D excess
Hypercalcemia, loss of appetite. Seen in sarcoidosis because epitheloid histiocytes activate vitamin d.
126
Vitamin E
Tocopherol. Antioxidant that protects erythrocytes and membranes from free radical damage. Can enhance effects of warfarin
127
Vitamin E deficiency
Hemolytic anemia, acanthosis, can cause neurologic symptoms like B12 but without the megaloblastic anemia or increase in methylmalonate.
128
Vitamin K
Cofactor for the gamma carboxylation of glutamate on coagfactors. Created by intestinal flora.
129
Vitamin K deficiency
Neonatal hemorrhage with increased PT and PTT but normal bleeding time. Not in breast milk.
130
Zinc
Essential cofactor. Deficiency has delayed wound healing, hypogonadism, decrease in hair.
131
Zinc
Essential cofactor. Deficiency has delayed wound healing, hypogonadism, decrease in hair.
132
Ethanol metabolism
EtOH turned into acetaldehyde by alcohol dehydrogenase (with NAD), in cyto. In mito, acetaldehyde turned into acetate by acetaldehyde dehydrogenase.
NAD is limiting reagent. Zero order kinetics.
Increases NADH to NAD ratio, causing pyruvate to lactate (lactic acidosis), oxaloacetate to malate (decreases gluconeogenesis -- hypoglycemia), g3p makes triglycerides.
Causes ketoacidosis and lipogenesis.
133
Fomepizole
blocks alcohol dehydrogenase. Antidote for methanol/ethylene glycol poisoning.
134
Disulfiram
Blocks acetaldehyde dehydrogenase, leads to hangover symptoms
135
Kwashiorkor
Protein malnutrition leads to edema, fatty change of liver. Anemia
136
Marasmus
Total calorie malnutrition. Muscle wasting loss of fat.
137
Marasmus
Total calorie malnutrition. Muscle wasting-- loss of fat.
138
Vitamin B12 deficiency
Megaloblastic anemia, hypersegmented neutrophils, paresthesias and subacute combined degeneration (degeneration of dorsal columns, LCSTs, due to abnormal myelin). Increases homocysteine and methylmalonic acid.
Pernicious anemia, diphyllobothrium latum.
139
Vitamin K
Cofactor for the gamma carboxylation of glutamate on coag factors. Created by intestinal flora.
140
Disulfiram
Blocks acetaldehyde dehydrogenase in mitochondria, leads to hangover symptoms
141
Alkaptonuria (ochonosis)
benign Deficiency of homogentisate oxidase in the tyrosine degradation pathway. Build up of homogentisic acid. Causes dark connective tissue, brown sclerae, urine turns black.
May have debilitating arthralgias (homogentisic acid is toxic to cartilage).
