Metabolism II Flashcards
ATP from NADH
2.5
ATP from FADH2
1.5
FADH2 electrons
to complex II of ETC
NADH electrons
to complex I of ETC
rotenone
inhibit complex I of ETC
antimycin A
inhibit complex III of ETC
cyaninde
inhibit complex IV of ETC
CO
inhibit complex IV of ETC
oligomycin
inhibit ATP synthase of ETC
-complex V
2,4 - dinitrophenol
uncoupling agent
illegal use of weight loss drug
aspirin and ETC
uncoupling agent
fever with aspirin OD
thermogenin
brown fat component -babies and hibernating animals
uncoupling agent - heat production
gluconeogenesis irreversible enzymes
pyruvate carboxylase
pyruvate > OAA
phosphoenolpyruvate carboxykinase
OAA > PEP
fructose 1,6 bisphoshatase (inhibited fructose 2,6 BP)
fructose 1,6 BP > fructose 6 P
glucose 6 phosphatase
glucose 6 P > glucose
gluconeogenesis
occur in liver
-maintain euglycemia during fasting
HMP shunt
produce NADPH
from glucose 6-P
G6PD - rate limiting enzyme
-inhibited by NADPH - feedback inhibition
rxns of HMP shunt
oxidative - irreversible - source of NADPH
nonoxidative - reversible
G6PD deficiency
X-linked recessive
NADPH necessary to keep glutathione reduced
low NADPH - hemolytic anemia
-poor RBC defense against oxidizing agents
fava beans, sulfa drugs, primaquin, TB drugs, infection
G6PD deficiency epidemiology
blacks, X-linked recessive
protection against malaria
heinz bodies
denatured Hb in RBC precipitate
in G6PD deficiency
bite cells
G6PD deficiency
macrophages in spleen remove heinz bodies from RBCs
essential fructosuria
benign - auto rec - often asymptomatic
deficient fructokinase
deficient fructokinase
essential fructosuria- often asymptomatic
fructose intolerance
deficient aldolase B - conversion fructose 1-P - accumulates
-auto rec
sx after have fructose or sucrose (glucose + fructose)
hypoglycemia, jaundice, cirrhosis, vomiting
avoid with fructose intolerance
fructose
sucrose (glucose and fructose)
aldolase B deficiency
galactokinase deficiency
galactitol accumulate
- mild condition
- auto rec
galactose in blood, infantile cataracts
classic galactosemia
absence of galactose 1 phosphate uridyltransferase
-auto rec
accumulation toxic substances - galactitol - in lens of eye
failure to thrive, jaundice, hepatomegaly, infant cataracts, intellect disability, E. coli sepsis
tx - no galactose or lactose (galactose and glucose)
deficiency in galactose 1 P uridyltransferase
classic galactosemia
accumulation of galactitol - lens of eye
sorbitol
alcohol counterpart of glucose
enzyme - aldose reductase - glucose > sorbitol
then convert sorbitol > fructose (sorbitol DH)
deficiency sorbitol DH
no sorbitol > fructose
-sorbitol accumulation
osmotic damage - cataract, retinopathy, peripheral neuropathy
high blood levels of galactose
result in conversion to osmotically active galactitol
aldose reductase
lactase deficiency
function on brush border - digest lactose > glucose and galactose
primary - age dependent decline after childhood
secondary - loss brush border - gastroenteritis, autoimmune
congenital - rare - defective gene
bloating, cramps, flatulence, osmotic diarrhea, decreased stool pH, hydrogen increase in breath lactose tolerance test
lactase deficiency
protein AAs
only L-amino acids
ketogenic AAs
leucine
lysine
acidic AAs
aspartic acid
glutamic acid
negative charge at body pH
basic AAs
arginine
lysine
histidine - no charge at body pH
AAs in histones
positive charge arginine and lysine
bind negatively charged DNA
required periods of growth
Arg and His
gluconeogenic AAs
methionine
valine
histidine
excess nitrogen
converted to urea - excreted by kidney
ammonia transport
alanine and glutamate
glutamate
converted to a-KG
amino acid
converted to a-ketoacid
