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Biochem: metabolism Flashcards

1
Q

What processes take place in the mitochondria?

A

fatty acid (B) oxidation
acetyl CoA production
TCA production
oxidative phosphorylation

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2
Q

What processes take place in the cytoplasm?

A 
glycolysis
fatty acid synthesis
HMP shunt
protein synthesis (RER)
steroid synthesis (SER)
cholesterol synthesis
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3
Q

What processes take place in the cytoplasm and mitochondria?

A

heme synthesis
urea cycle
gluconeogenesis

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4
Q

What processes is NADPH used for?

A

anabolic processes
respiratory burst
P-450
glutathione reductase

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5
Q

What produces NADPH?

A

HMP shunt

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6
Q

What glycolytic enzymes require ATP?

A

hexokinase/glucokinase

phosphofructokinase**(rate limiting)

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7
Q

What glycolytic enzymes produce ATP?

A

phosphoglycerate kinase

pyruvvate kinase

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8
Q

What happens to FBPase2 and PFK 2 in the fasting state?

A

^glucagon, ^FBPase 2, less glycolysis

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9
Q

What happens to FBPase 2 and PFK 2 in the fed state?

A

^insulin, ^PFK 2, more glycolysis

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10
Q

What are the cofactors in the pyruvate dehydrogenase complex?

A
  1. pyrophosphate (B1;TPP)
  2. FAD (B2/riboflavin)
  3. NAD (B3/niacin)
  4. CoA (B5/pantothenic acid)
  5. Lipoic acid
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11
Q

What enzyme requies the same cofactors as PDH complex?

A

a KG dehydrogenase complex

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12
Q

Sx of arsenic poisoning?

A

inhibits lipoic acid

vomiting, rice water stools, garlic breath

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13
Q

What activates PDH complex?

A

exercise
^ NAD+/NADH ratio
^ADP
^Ca2+

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14
Q

What are sx of PDH complex deficiency?

A

neurologic defect

lactic acidosis

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15
Q

Tx for PDH complex def?

A

^intake of ketogenic nutrients (high fat, lysine, leucine)

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16
Q

What causes PDH complex def?

A

mutation in X linked gene for E1-a subunit of PDC

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17
Q

What reaction does PDH complex catalyze?

A

pyruvate –> acetyl coA

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18
Q

What does 2,4 dinitrophenol do?

A

uncouple oxidative phosphorylation

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19
Q

What inhibits complex 1?

A

rotenone

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20
Q

What inhibits comlex III?

A

antimycin A

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21
Q

What inhibits complex IV?

A

cyanide

CO

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22
Q

What inhibits complex V?

A

oligomycin

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23
Q

What are the elctron transport inhibitors?

A

rotenone, cyanide, antimycin A, CO

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24
Q

What is an ATP synthesis inhibitor?

A

oligomycin

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25
What are some uncoupling agents?
2,4-DNP Aspirin (fevers occur w/OD) Thermogenin (in brown fat)
26
What are the irreversible enzymes of gluconeogenesis? | Location?
pyruvate carboxylase-mito PEP carboxykinase-cyto Fructose 1,6 bisphosphatase-cyto Glucose 6 phosphatase-ER
27
Where does the HMP shunt take place (cell types)?
lactating mammary glands, liver, adrenal cortex, RBCs
28
What is glutathiones role?
detoxifies free radicals and peroxides
29
What causes essential fructosuria? Inheritance? Sx?
defect in fructokinase AR benign, asymptomatic fructose appears in blood and urine
30
What causes fructose inotlderance? inheritance? Sx?
deficiency of aldolase B AR leads to decrease in available phosphate-->inhibition of glycogenolysis and gluconeogenesis hypoglycemia, jaundice, cirrhosis, vomiting
31
How is fructose intolerance treated?
decrease intake of fructose and sucrose (glucose + fructose)
32
What is seen with galactokinase deficiency? inheritance?
galactitol accumulates if galactose is present in diet galactose appears in blood and urine, infantile cataracts, may initially present as failure to track objects or develop a social smile mild AR
33
What is seen in classic galactosemia? inheritance?
absence of galactose-1-phosphate uridyltransferase accumulation of toxic substances (including galactitol in the lens) PO4 deficiency failure to thrive, jaundice, hepatomegaly, infantile cataracts, MR AR
34
How is classic galactosemia treated?
excluse galactose and lactose (galactose+glucose) from diet
35
What converts glucose to its alcohol component? What is the alcohol called? Why do cells do this?
aldose reductase sorbitol done as alternative method of trapping glucose in cells
36
What can excess sorbitol cause?
osmotic damage (cataracts, retinopathy, peripheral neuropathy)
37
What enzyme converts sorbitol to fructose?
sorbitol dehydrogenase
38
What cell types are missing sorbitol dehydrogenase?
schwann cells, retina, kidneys
39
What organs have both aldose reductase and sorbitol dehydrogenase?
liver, lens, ovaries, seminal vesicles
40
Where is lactase located?
brush border enzyme
41
What are the essential amino acids?
glucogenic: met, val, his glucogenic/ketogenic: ile, phe, thr, trp ketogenic: leu, lys
42
What is seen with galactokinase deficiency? inheritance?
galactitol accumulates if galactose is present in diet galactose appears in blood and urine, infantile cataracts, may initially present as failure to track objects or develop a social smile mild AR
43
What is seen in classic galactosemia? inheritance?
absence of galactose-1-phosphate uridyltransferase accumulation of toxic substances (including galactitol in the lens) PO4 deficiency failure to thrive, jaundice, hepatomegaly, infantile cataracts, MR AR
44
How is classic galactosemia treated?
excluse galactose and lactose (galactose+glucose) from diet
45
What converts glucose to its alcohol component? What is the alcohol called? Why do cells do this?
aldose reductase sorbitol done as alternative method of trapping glucose in cells
46
What AAs carry ammonium?
alanine | glutamate
47
What enzyme converts sorbitol to fructose?
sorbitol dehydrogenase
48
What cell types are missing sorbitol dehydrogenase?
schwann cells, retina, kidneys
49
What organs have both aldose reductase and sorbitol dehydrogenase?
liver, lens, ovaries, seminal vesicles
50
Where is lactase located?
brush border enzyme
51
What are the essential amino acids?
glucogenic: met, val, his glucogenic/ketogenic: ile, phe, thr, trp ketogenic: leu, lys (arg and his are also required during periods of growth)
52
what are the acidic amino acids?
asp, glu (neg charge at body pH)
53
what are the basic amino acids?
arg, lys, his
54
Where is the enzyme ornithine transcarbamoylase located?
mitochondria
55
urea cycle order of compounds (ordinarily careless crappers are also frivolous about urination)
``` ornithine/carbamoly phosphate citrulline/aspartate arginosuccinate arginine/fumarate urea ```
56
What AAs carry ammonium?
alanine | glutamate
57
What are sx of ammonia intoxication?
``` tremor (asterixis) slurred speech somnolence vomiting cerebral edema blurring of vision ```
58
What are the 2 categories of causes for hyperammonemia?
acquired (liver disease) | hereditary (urea cycle enzyme deficiencies)
59
What does excess NH4 inhibit?
TCA because it depletes a ketoglutarate
60
How do you treat hyperammonemia?
limit protein in diet benzoate or phenylbutryate to decrease ammonia levels (bind AA and lead to excretion) lactulose
61
What are findings of OTC deficiency?
increased orotic acid in blood and urine, decreased BUN, sx of hyperammonemia usually presents first few days of life
62
How is OTC def inherited? Why does this cause problems?
X linked recessive | interferes with the body's ability to eliminate ammonia
63
What can happen to babies whose mothers have PKU?
(when mom isn't getting proper dietary therapy during pregnancy) microcephaly, MR, growth retardation, congenital heart defects
64
What is made from tryptophan?
Niacin/NAD/NADP | serotonin, melatonin
65
What is made from histidine?
histamine
66
What is made from glycine?
porphyrin/heme
67
What causes albinism?
``` deficiecny of tyrosinase (cant make melanin, AR) defective tyrosine transporters (decreased amt of tyrosine and thus melanin) lack of migration of neural crest cells **ocular albinism is X linked recessive ```
68
What is made from glutamate?
GABA | glutathione
69
What are the product of MAO/COMT?
HVA (from dopamine) VMA (from NE) Metanephrine (from Epi)
70
What causes Phenylketonuria?
decreased phenylalanine hydroxylase or decreased tetrahydrobiopterin cofactor
71
What is essential in PKU? What must be avoided? What is in the urine?
tyrosine avoid phenylalanine phenylketones
72
What are sx of PKU?
MR, growth retardation, seizures, fair skin, eczema, musty body odor
73
What can happen to babies whose mothers have PKU?
(when mom isn't getting proper dietary therapy during pregnancy) microcephaly, MR, growth retardation, congenital heart defects
74
What causes alkaptonuria (ochronosis)?
deficiency of homogentisic acid oxidase (can't degrade tyrosine to fumarate)
75
What are sx of alkaptonuria?
benign dark connective tissue, brown pigmented sclera, urine turns black on prolonged exposure to air may have debilitating arthralgias (homogentisic acid toxic to cartilage)
76
What drug blocks DOPA decarboxylase in the periphery?
carbidopa
77
What causes albinism?
``` deficiecny of tyrosinase (cant make melanin, AR) defective tyrosine transporters (decreased amt of tyrosine and thus melanin) lack of migration of neural crest cells **ocular albinism is X linked recessive ```
78
What are sx of hartnup disease?
pellagra (bc can't make B3)-dermatitis, diarrhea, dementia
79
What are findings in homocystinuria?
increased homocysteine in urine | MR, osteoporosis, tall stature, kyphosis, lens subluxation, atherosclerosis (stroke and MI)
80
What causes cystinuria?
AR defect of renal tubular amino acid transporter for cysteine, ornithine, lysine and arginine in the PCT of kidney
81
What are sx of cystinuria?
precipitation of hexagonal crystals and renal staghorn calculi
82
Tx for cystinuria?
hydration and urinary alkalinization (acetazolamide)
83
What is cystine?
2 cysteines connected by a disulfide bond
84
What are the branched amino acids?
isoleucine, leucine, valine
85
What causes maple syrup urine disease?
can't degrade branched amino acids due to decreased a-ketoacid dehydrogenase AR
86
What are sx of maple syrup urine disease?
severe CNS defects, MR, death urine smells like maple syrup increased a-ketoacids in the blood, esp leucine
87
What is hartnup disease?
AR disorder with defective neutral amino acid transporter on renal and intestinal epithelia cells, leads to tryptophan excretion in urine and decreased absorption in the gut unable to make B3
88
Sx of type III/cori's disease?
milder for of type I w/ normal blood lactate levels (so hypoglycemia and hepatomegaly but not as bad as von gierke's) gluconeogenesis intact
89
What do both glucagon and epinephrine upregulate in cells? What does this cause?
Adenylyl cyclase/cAMP | leads to activation of PKA and glycogen phophyrlase kinase (glycogenolysis)
90
What happens when insulin tyrosine kinase dimeraizes?
activates protein phosphatase (inactivates glycogen phosphorylase & glycogen phosphorylase kinase)
91
What activates glycogen phospholyase kinase in muscles?
Ca2+ and calmodulin | coordinates glycogenolysis with muscle activity
92
What types of linkages are found in glycogen?
a1,6 a1,4
93
How are all the glycogen storage disease inherited?
AR
94
Sx of type I/Von Gierke's disease?
severe fasting hypoglycemia increased glycogen in liver increased blood lactate hepatomegaly
95
Enzyme deficient in von gierke's?
Glucose 6 phosphatase
96
Sx of type II/Pompes?
cardiomegaly and systemic findings leading to early death | heart, liver and muscle damage
97
Enzyme deficient in pompes?
lysosomal a-1,4 glucosidase (acid maltase)
98
Sx of type III/cori's disease?
milder for of type I w/ normal blood lactate levels (so hypoglycemia and hepatomegaly but not as bad as von gierke's)
99
Enzyme deficient in cori's?
debranching enzyme (a-1,6 glucosidase)
100
Type V/McArdle's sx?
increase glycogen in muscle but can't break it down | leads to painful muscle crames and myoglobinuria (muscle cells swell and lyse) with strenuous exercise
101
What enzyme deficient in McArdle's?
skeletal muscle glycogen phosphorylase
102
What enzyme is deficient in Fabry's disease? What accumulates? Inheritance?
a-galactosidase A ceramide trihexoside XR
103
What are sx of Fabry's disease?
peripheral neuropathy of hands/feet angiokeratomas (esp on lower abdomen, buttocks and groin) cardiovascular/renal disease
104
Sx of hurler's s/o?
``` developmental delay gargoylism airway obstruction corneal clouding hepatosplenomegaly ```
105
What are sx of Gaucher's disease?
hepatosplenomegaly, aseptic necrosis of femur, bone crises, Gaucher's cells (MOs that look like crumpled tissue paper)
106
What enzyme is deficient in Niemann-Pick disease? What accumulates? Inheritance?
Sphingomyelinase Sphingomyelin AR
107
What are sx of Niemann-Pick disease?
progressive neurodegeneration, hepatosplenomegaly, cherry red spot, foam cells worsening of intellectual disability
108
What enzyme is deficient in Tay Sachs? What accumulates? Inheritance?
Hexosaminidase A GM2 ganglioside AR
109
What are sx of Tay Sachs disease?
progressive neurodegeneration, developmental delay, cherry red spot, lysosomes w/onion skin NO hepatosplenomegaly
110
What enzyme is deficient in Krabbe's disease? What accumulates? Inheritance?
Galactocerebrosidase Galactocerebrosde (psychosine) AR
111
Waht are sx of Krabbe's disease?
peripheral neuropathy, developmental delay, optic atrophy, globoid cells
112
What enzyme is deficient in metachromic leukodystrophy? What accumulates? Inheritance?
arylsulfatase A cerebroside sulfate AR
113
What enzyme is deficient in Hurler's syndrome? What accumulates? Inheritance?
a-L-iduronidase heparan sulfate, dermatan sulfate AR
114
What is providing fuel after fed state?
glycolysis and aerobic respiration | insulin stimulates storage of lipids, porteins and glycogen
115
What enzyme is deficient in Hunter's syndrome? What accumulates? Inheritance?
iduronate sulfatase heparan sulfate, dermatan sulfate XR
116
What are sx of Hunter's s/o?
mild hurler's + aggressive behavior | NO corneal clouding
117
Which of the lysosomal storage disease are Ashkenazi jews and increased risk for?
Tay SAchs Niemann Pick Gauchers
118
What happens in carnitine deficiency?
inability to transport LCFAs into the mitochondria leads to toxic accumulation weakness, hypotonia, hypoketoia hypoglycemia
119
What happens with acyl CoA dehydrogenase deficiency?
increased dicarboxylic acids | decreased glucose and ketones
120
What are fatty acids and amino acids metabolized to in the liver (during starvation)?
acetoacetate and B hydroxybutyrate (ketones)
121
What is depleted during DKA?
oxaloacetate-->no substrate for gluconeogenesis | have to make ketone bodies instead
122
How many calories are in 1g of protein or carbohydrate?
4 kcal
123
How many calories are in 1g of fat?
9 kcal
124
What is providing fuel after fed state?
glycolysis and aerobic respiration
125
What provides fuel in fasting state (between meals)
``` hepatic glycogenolysis (major), hepatic gluconeogenesis/adipose release of FFA (minor) glucagon and adrenaline sitmualte use of fuel reserves ```
126
What does hormone sensitive lipase do?
degrade TG stores in adipocytes
127
What cell type cannot use ketones for fuel?
RBCs
128
What does cholesterol ester transfer protein (CETP) do?
mediate transfer of cholesterol esters to other lipoprotein particles
129
After 3 days of starvation what is maintaining blood glucose?
adipose stores/ketone bodies | after adipse depleted-protein degradation (can lead to organ failure and death)
130
What determines length of starvation survival time?
amount of adipose stores (fatties FTW)
131
What reaction does HMG CoA reductase stimulate?
HMG CoA to mevalonate
132
What esterified plasma cholesterol?
lecithin-cholesterol acyltransferase
133
What does ApoB100 do? What is it a part of?
binds LDL-R | VLDL, IDL, LDL
134
What does lipoprotein lipase do?
degrade TG circulating in chylomicrons and VLDLs
135
What does hepatic TG lipase do?
degrade TG remaining in IDL
136
What does hormone sensitive lipase do?
degrade TG stores in adipocytes
137
What does lecithin-cholesterol acyltransferace (LCAT) do?
catalyze esterification of cholesterol
138
What does cholesterol ester transfer protein (CETP) do?
mediate transfer of cholesterol esters to other lipoprotein particles
139
What does ApoE do? What is it a part of?
mediates remnant uptake | chylomicron/chylomicron remnant, VLDL, IDL, HDL
140
What does LDL do?
deliver hepatic cholesterol to peripheral tissues formed by modifiying IDL in peripheral tissue taken up by target cells via receptor meidated endocytosis
141
What does ApoC-II do? What is it a part of?
lipoprotein lipase cofactor | chylomicron, VLDL, HDL
142
What does ApoB48 do? What is it a part of?
mediates chylomicron secretion | chylomicron/chylomicron remnant
143
What does ApoB100 do? What is it a part of?
binds LDL-R | VLDL, IDL, LDL
144
What carries the majority of cholesterol?
LDL and HDL
145
What transports cholesterol from liver to tissues?
LDL
146
What transports cholesterol from periphery to liver?
HDL
147
What do chylomicrons do?
deliver dietary TGs to peripheral tissue, delivers cholesterol to liver (remnants) secreted by intestinal epithelial cells
148
What does VLDL do?
delivers hepatic TGs to peripheral tissue | secreted by liver
149
What does IDL do?
formed by degradation of VLDL | delivers TGs and cholesterol to liver
150
What does LDL do?
deliver hepatic cholesterol to peripheral tissues formed by modifiying IDL in peripheral tissue taken up by target cells via receptor meidated endocytosis
151
What does HDL do?
mediates reverse choelsterol transport from periphery to liver repository for apoC and apoE (needed for chylomicron and VLDL metabolism) secreted from liver and intestine
152
What is increased in blood with 1-hyper-chylomicronemia
chylomicrons, TG, cholesterol
153
What causes 1-hyper chylomicronemia/
AR lipoprotein lipase deficiency or altered apolipoprotein C-II
154
Sx of 1-hyper chylomicronemia?
pancreatitis, hepatosplenomegaly, eroptive/pruritic xanthomas NO increased risk for atherosclerosis
155
What is increased in blood for IIa familial hyper cholesterolemia?
LDL, cholesterol
156
What causes IIa familial hypercholesterolemia?
AD deficiency or decreased LDL receptors
157
Sx of IIa familial hypercholesterolemia/
accelerated atherosclerosis, tendon xanthomas, corneal arcus
158
What is elevated in blood for IV-hypertriglyceridemia?
VLDL, TG
159
What causes IV-hypertriglyceridemia?
AD hepatic overproduction of VLDL
160
Sx of IV-hypertriglyceridemia?
pancreatitis
161
What causes abetalipoproteinemia?
AR mutation in microsomal triglyceride transfer protein (MTP) gene decreased B48 and B100 decreased chylomicron and VLDL synthesis and secretion l
162
What does biopsy show for abetalipoproteinemia?
lipid accumulation in enterocytes due to inability to export absorbed lipid as chylomicrons
163
Sx of abetalipoproteinemia?
failure to thrive, steatorrhea, acanthocytosis, ataxia, night blindness
164
What is the treatment for abetalipoproteinemia?
vitamin E

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