Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Step I > metabolism > Flashcards

metabolism Flashcards

(156 cards)

Start Studying
1
Q

mitochondria

A 
beta oxidation
acetyl CoA production
TCA cycle
ox phos
ketogenesis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

cytoplasm

A 
glycolysis
fatty acid synthesis
HMP shunt
protein synthesis (RER)
steroid synthesis (SER)
cholesterol synthesis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

both mito and cyto

A

heme synthesis
urea cycle
gluconeogenesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

kinase

A

uses ATP to add phosphate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

phosphorylase

A

adds inorganic phosphate onto substrate w/o ATP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

phosphatase

A

removes phosphate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

dehydrogenase

A

catalyzes oxidation-reductions rxn

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

hyrdoxylase

A

adds hydroxyl (-OH)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Carboxylase

A

transfers CO2 w/help of biotin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

mutase

A

relocates fnx grp w/in molecule

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

glycolysis rate-limiting step enzyme

A

phosphofructokinase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

gluconeogenesis rate-limiting step enzyme

A

fructose 1,6 bisphosphatase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

TCA cycle rate-limiting step enzyme

A

isocitrate dehydrogenase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

glycogenesis rate-limiting step enzyme

A

glycogen synthase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

glycogenolysis rate-limiting step enzyme

A

glycogen phosphorylase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

HMP shunt rate-limiting step enzyme

A

carbamoyl phosphatate synthetase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

De novo pyrimidine synthesis rate-limiting step enzyme

A

carbamoyl phosphate synthetase II

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

de novo purine synthesis rate-limiting step enzyme

A

glutamine-PRPP amidotransferase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

ureas cycle rate-limiting step enzyme

A

carbamoyl phosphate synthetase I

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

FA synthesis rate-limiting step enzyme

A

acetyl-CoA carboxylase (ACC)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

FA oxidation rate-limiting step enzyme

A

carnitine acyltransferase I

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

ketogenesis

A

HMG-CoA synthase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Cholesterol synthesis

A

HMG-CoA reductase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

arsenic

A 
glycolysis -> zero net ATP
inhibits lipoic acid
vomiting
rice-water stool
garlic breath
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
NAD
electron acceptor | usually catabolic processes
26
NADPH
electron acceptor | used in anabolic processes
27
hexokinase
phosphorylation of glucose - G6P 1st step of glycolysis and glycogen synthesis expressed in most tissues except liver and beta cells higher affinity/lower Km then glucokinase lower capacity/Vmax then glucokinase not induced by insulin inhibited by G6P gene mutation NOT associated w/ MODY
28
glucokinase
``` phosphorylation of glucose - G6P 1st step of glycolysis and glycogen synthesis expressed in liver and beta cells lower affinity/higher Km then hexokinase higher capacity/Vmax then hexokinase IS induced by insulin NOT inhibited by G6P gene mutation IS associated w/ MODY ```
29
which glycolysis enzymes require ATP?
hexokinase/glucokinase | phosphofructokinase-1 (RLS)
30
which glycolysis enzymes produce ATP?
phosphoglycerate kinase | pyruvate kinase
31
fructose 2.6 bisphosphonate
FBPase-2 and PFK-2 are same enzyme whose direction is reversed by phosphorylation via PKA fasting: increased glucagon -> increased cAMP -> increased PKA -> increased FBPase 2 -> less glycolysis or gluconeogenesis fed: increased insulin -> decreased cAMP -> decreased PKA -> increased PFK-2 -> more glycolysis and less gluconeogenesis
32
pyruvate dehydrogenase complex
mito enzyme complex linking glycolysis and TCA cycle (active in fed state) pyruvate + NAD + CoA -> acetyl CoA + CO2 + NADH activated by exercise which increases NAD, ADP, and Ca
33
pyruvate dehydrogenase complex deficiency
x-linked build-up of pyruvate -> lactate and alanine neuro defects, lactic acidosis, increased serum alanine starting in infancy
34
Tx of pyruvate dehydrogenase complex deficiency
increase intake of ketogenic nutrients high fat lysine and leucine
35
pyruvate can become what?
alanine (via ALT for Cahill cycle) oxaloacetate (via PC for TCA cycle or gluconeogenesis) acetyl-CoA (via PDH for TCA cycle) Lactate ( via LDH for Cori Cycle)
36
TCA cycle
``` Citrate Is Krebs Starting Substrate For Making Oxaloacetate citrate isocitrate alpha-KG succinyl CoA succinate fumarate malate oxaloacetate ```
37
rotenone
blocks complex I of ETC
38
antimycin A
blocks complex III of ETC
39
cyanide
blocks complex IV of ETC
40
CO
blocks complex IV of ETC
41
Oligomycin
blocks complex V(ATP synthase) of ETC
42
uncoupling agents
2,4 dinitrophenol aspirin thermogenin in brown fat
43
gluconeogenesis irreversible enzymes
``` Pathway Produces Fresh Glucose pyruvate carboxylase phosphoenolpyruvate carboxykinase fructose 1,6 bisphosphatase glucose 6 phosphate ```
44
pyruvate carboxylase
in mito | pyruvate -> oxaloacetate
45
phosphoenolpyruvate carboxykinase
in cytosol | oxaloacetate -> phophoenolpyruvate
46
fructose 1,6 bisphosphatase
in cytosol | fructose 1,6 bisphosphate -> fructose 6 phosphate
47
glucose 6 phosphatase
in ER | G6P -> glucose
48
HMP shunt/pentose phosphate pathway
provides source of NADPH from G6P yields ribose for nucleotide synthesis NO ATP used or produces occurs in lactating mammary glands, liver, adrenal Cx
49
oxidative rxn
use NADP+ create and inhibited by NADPH produce CO2 irreversible
50
nonoxidative rxns
reversible
51
G6PD deficiency
``` XR MC human enzyme deficiency more prevalent in blacks increases malarial resistance G6PD required to replenish NADPH which is needed for glutathione reductase ```
52
G6PD presenation
hemolytic anemia following favao beans, sulfonamides, primaquine, atnituberculosis drugs, or infection
53
G6PD PBS
heinz bodies- denatured hemoglobin precipitates in RBCs bite cells- results from phagocytic removal of Heinz bodies 'Bite into some Heinz ketchup on fava beans'
54
essential fructosuria
defect in fructokinase- AR benign asymptomatic condition since fructose not tapped in cells fructose in blood and urine
55
fructose intolerance
AR deficiency of aldolase B fructose-1-phosphate accumulates -> decreased bioavailability of phosphate -> inhibition of glycogenolysis and gluconeogenesis urine dip stick neg
56
symptoms of fructose intolerance
hypoglycemia, jaundice, cirrhosis, vomiting
57
Tx of fructose intolerance
decrease fructose and sucrose in diet
58
galactokinase deficiency
galactitol accumulates if galactose in diet mild AR
59
symptoms of glactokinase deficiency
galactose in blood and urine infantile cataracts failure to track objects or develop a social smile
60
classic galactosemia
absence of galactose-1-phosphate uridyltransferase AR accumulation of toxic substances in lens of eye
61
symptoms of classic galatosemia
``` failure to thrive jaundice hepatomegaly infantile cataracts intellectual disability E. coli sepsis ```
62
Tx of classic galactosemia
exclude galactose and lactose from diet
63
sorbitol
glucose can be converted to sorbitol vis aldose reductase | can then be converted to fructose via sorbitol dehydrogenase
64
sorbitol dehydrogenase deficiency
intracellular sorbitol accumulation -> osmotic damage (cataracts, retinopathy, peripheral neuropathy) seen in DM
65
primary lactase deficiency
age dependent decline after childhood | common in asian, african, or native american descent
66
secondary lactase deficiency
loss of BBE d/t gastroenteritis, autoimmune disease, etc...
67
congenital lactase deficiency
rare | defective gene
68
lactase deficiency
lactase usually breaks lactose into glucose and galactose stool decreased pH breath shows increased hydrogen
69
symptoms of lactase deficiency
bloating, cramps, flatulence, osmotic diarrhea
70
glucogenic essential AAs
methionine (Met) Valine (Val) Histadine (His)
71
gluconeogenic/ketogenic essential AAs
isoleucine (Ile) pheylalanine (Phe) threonine (Thr) tryptophan (Trp)
72
ketogenic essential AAs
leusine (Leu) | lysine (Lys)
73
acidic AAs
``` aspartic acid (Asp) Glutamic acid (Glu) ```
74
basic AAs
``` arginine (Arg) lysine (Lys) histadine (His) Arg and Lys in histones Arg and His needed during growth ```
75
urea cycle
excess nitrogen converted to urea Ordinarily Careless Crappers Are Also Frivolous About Urination Ornithine + Carbamoyl phosphate -> Citrulline Citrulline + Aspartate -> Arginosuccinate -> Fumarate +Arginine Arginine -> Urea +Ornithine
76
hyperammonemia
excess NH4 -> depletes alpha ketoglurarate -> inhibits TCA cycle tremor, slurring of speech, somnolence, vomiting, cerebral edema, blurring of vision
77
Tx of hyperammonemia
limit protein in diet lactulose -> acidifies GI tract to trap NH4 Rifaximin -> decrease colonic ammoniagenic bacteria benzoate or phelbutyrate -> bind AAs
78
N-acetylglutamate synthase deficiency
required cofactor for carbamoyl phosphate snthetase I -> hyperammonemia presents in neonates as poorly regulated resp and body temp, poor feeding, developmental delay, intellectual disability
79
ornithine transcarbamylase deficiency
MC urea cycle disorder XR (other urea cycle disorders AR) usually presents first few days of life, but may be later excess carbamoyl phosphate is converted to orotic acid
80
findings in ornithine transcarbamylase deficiency
increased orotic acid in blood and urine decreased BUN symptoms of hyperammonemia NO megaloblastic anemia in contrast to orotic aciduria
81
pheylketonruia
AR d/t decreased phenylalanine hydroxylase or decreased tetrahydrobiopterin cofactor (malignant PKU) increased phenylalanine -> pheylketones in urine
82
findings of PKU
``` normal at birth b/c of maternal enzyme intellectual disability growth retardation seizures fair skin eczema musty body odor ```
83
Tx of PKU
decreased phenylalanine and increased tyrosine in diet tetrahydrobiopterin supplementation cannot eat aspartame
84
PKU memory jog
disorder of aromatic aa meta -> musty body odor
85
maternal PKU
lack of proper dietary therapy during prego | findings in infant -> microcephaly, intellectual disability, growth retardation, congenital heart defects
86
maple syrup urine disease
AR | blocked degresation of brr aa (isoleucine, leucine, and valine) d/t decreased alpha ketoacid dehydrogenase
87
symptoms of maple syrup urine disease
severe CNS defects intellectual disability death urine smells like maple syrup/burnt sugar
88
Tx of maple syrup urine disease
restriction of isoleucine, leucine, and valine in diet | thiamine supplementation
89
maple syrup urine disease pneumonic
I Love Vermont maple syrup from maple trees w/Brr
90
Alkaptnuria/ochronosis
AR, usually benign deficiency of homogentisate oxidase in degradative pathway of tyrosine to fumarate-> pigment forming homogentisic acid accumulates in tissues
91
alkaptnuria/ochronosis findings
dark CT brown pigmented sclera urine turns black on prolonged exposure to air may have debilitating arthralgias
92
homocystinuria
``` all types AR excess homocysteine in urine intellectual disability osteoporosis marfanoid habitus kyphosis lens subluxation thrombosis and atherosclerosis ```
93
cystinuria
``` AR, common defect of renal PCT and intestinal aa transport prevents reabsorption of COLA hexagonal cystine stones urinary cyanide-nitroprusside test Dx ```
94
COLA
cysteine ornithine lysine arginine
95
cystinuria Tx
chelating (pencicillamine) | hydration
96
glycogen storage diseases
``` all AR Very Poor Carb Meta Von Gierke's disease (type I) Pompe (type II) Cori (type III) McArdle (type V) ```
97
Von Gierke's what enzyme?
G6P | AR
98
Von Gierke's findings
``` severe fasting hypoglycemia big increase in glycogen in liver increased blood lactate increased TGs increased uric acid hepatomegaly ```
99
Von Gierke's Tx
frequent oral glucose/cornstarch | avoid fructose and galactose
100
Pompe what enzyme?
lysosomal alpha 1,4 glucosidase (acid maltase) | AR
101
Pompe findings
cardiomegaly/hypertrophic cardiomyopathy exercise intolerance systemic findings -> early death
102
Pompe acronym
Pompe trashes the Pumps (heart, mm, liver)
103
cori disease what enzyme?
debrr/alpha-1,6 glucosidase
104
cori disease findings
``` milder form of type I w/normal blood lactate levels, gluconeogenesis intact type I: severe fasting hypoglycemia big increase in glycogen in liver increased blood lactate increased TGs increased uric acid hepatomegaly ```
105
McArdles disease
increase glycogen in mm but mm cannot break it down -> painful mm cramps myoglobinuria w/strenuous exercise arrhythmia from electrolyte abnormalities blood glucose typically normal
106
McArdles enzyme
skeletal mmm glycogen phosporylase/myophosphorylase
107
McArdles Tx
vit B6 (pyridoxine)
108
Fabry disease
XR deficient alpha galactosidase accumulates ceramide trihexoside
109
fabry findings
peripheral neuropathy hands/feet angiokeratomas cardiovascular/renal disease
110
gaucher disease
AR deficient glucocerebrosidase accumulates glucocerebroside most common lysosomal storage disease
111
gaucher findings
``` hepatosplenomegaly pancyotpenia osteoporosis aseptic necrosis of femur bone crises gaucher cells (lipid laden macros resembling crumpled tissue paper) ```
112
gaucher Tx
recombinant glucocerebrosidase
113
Niemann pick disease
AR deficient spingomyelinase accumulates spingomyelin
114
niemann pick findings
progressive neurodegeneration hepatosplenomegaly foam cells cherry red spot on macula
115
tay-sachs disease
AR deficient hexosamindase A accumates GM2 ganglioside
116
tay sachs findings
``` progressive neurodegeneration developmental delay cherry red spot on macula lysosomes w/onion skin NO hepatosplenomegaly ```
117
krabbe disease
AR deficient galactocerebrosidase accumulates galactocerebroside and psychosine
118
krabbe disease findings
peripheral neuropathy developmental delay optic atrophy globoid cells
119
metachromatic leukodystrophy
AR deficient in arylsulfatase A accumulates cerebroside sulfate
120
metachromatic leukodystrophy
central and peripheral demyelination w/ataxia and dementia
121
hurler syndrome
AR deficient alpha-L-iduronidase accumulates heparan sulfate
122
hurler syndrome findings
``` developmental delay gargoylism airway obstruction corenal clouding hepatosplenomegaly ```
123
hunter syndrome
XR deficient iduronate sulfatase accumulates heparan sulfate and dermatan sulfate
124
hunter syndrome findings
mild hurler + aggression | no corneal clouding
125
Niemann picks acronym
No man picks his nose w/his sphinger (sphingomyelinase)
126
Tay-sachs acronym
Tay-SaX lacks heXosaminidase
127
Hunters syndrome acronym
Hunters see clearly (no corneal clouding) and aggressively aim for the X (XR)
128
which lysosomal diseases assocaites w/Ashkenazi Jews
Tay-Sachs Niemann picks gaucher
129
systemic primary carnitine deficiency
defect in transport of LCFA into mito toxic accumulation weakness, hyptonia, and hypoketotic hypoglycemia
130
medium chain acryl-CoA dehydrogenase deficiency
AR cannot break down FAs into Acetyl CoA accumulation of 8-10 carbon fatty acyl carnitines in blood hypoketotic hypoglycemia
131
medium chain acryl-CoA dehydrogenase deficiency presentation
infancy- early childhoos vomiting, lethargy, seizures, coma, liver dysfunction may cause sudden death, must avoid fasting
132
1g protein or fat
4kcal
133
1g fat
9kcal
134
1g alcohol
7kcal
135
cholesterol synthesis RLE
HMG-CoA reductase (induced by insulin) converts HMG-CoA -> mevalonate
136
LPL
lipoprotein lipase degradation of TGs circulating in chylomicrons and VLDLs on vascular endo surface
137
HL
hepatic lipase | degradation of TGs remaining in IDL
138
Hormone-sensitive lipase
degradation of TGs stored in adipocytes
139
LCAT
catalyzes esterification of cholesterol
140
apolipoprotein E
mediates remnant uptake | found on chylomicron, chylomicron remnant, VLDL, IDL, HDL
141
apoplipoprotein A-I
activates LCAT | found on chylomicron and HDL
142
apoplipoprotein C-II
lipoprotein lipase cofactors | found on chylomicron, VLDL, HDL
143
apoplipoprotein B-48
mediates chylomicron secretion | found on chylomicron, chylomicron remanant
144
apoplipoprotein B-100
binds LDL R | found on VLDL, IDL, LDL
145
LDL
transports cholesterol from liver to tissues
146
HDL
transports cholesterol from periphery to liver acts as repository for apoplipoprotein C and E (needed for chylomicron and VLDL meta) secreted both from liver and intestines alcohol increases synthesis
147
chylomicron
delivers dietary TGs to periphery delivers cholester to liver in form of chylomicron remnant secreted by intestinal epi
148
VLDL
delivers hepatic TGs to periphery | secreted by liver
149
IDL
formed in degradation of VLDL | delivers TGs and cholesterol to liver
150
LDL
delivers hepatic cholesterol to peripheral tissues formed by hepatic lipase modification of IDL in periphery taken up by target cells via R mediated endocytosis
151
familial dysplipidemia type I
hyper-chylomicronemia increased chylomicrons, TGs, and cholesterol in blood creamy layer in supernatant AR
152
path of familial dysplipidemia type I
lipoprotein lipase deficiency or altered apolipoprotein C-II causes pancreatitis, hepatosplenomegaly, eruptive/pruitic xanthoms no increased risk for atherosclerosis
153
familial dyslipidemia type IIa
familial hypercholesterolemia increased LDL, cholesterol AD
154
path of familial dyslipidemia type IIa
absent or defective LDL R heterozyogotes have cholesterol 300+ homozygotes have cholesterol 700+ (rare) accelerated atheroscloersis (MI
155
familial dyslipidemia type IV
familial hypertriglyceridemia increased VLDL, TG AD
156
path of familial dyslipidemia type IV
hepatic overproduction of VLDL | TGs >1000 can cause acute pancreatitis

Step I (22 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions