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First Aid Biochem > FA Biochem III > Flashcards

FA Biochem III Flashcards

1
Q

What fxn does glucokinase serve in the liver

A

phosphorylation excess glucose to sequester it, liver becomes blood glucose buffer

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2
Q

What are the reactants for glycolysis

A

glucose, 2Pi, 2ADP, 2NAD+

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3
Q

What are the products for glycolysis

A

2 pyruvate, 2ATP, 2NADH, 2H+, 2H2O

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4
Q

What two rxns in in glycolysis require ATP

A

glucose to G-6P, hexokinase and fructose-6P to fructose 1,6 BP phosphofructokinase-1

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5
Q

What substances inhibit phosphofructokinase-1

A

ATP, citrate

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6
Q

What substances induce phosphofructokinase

A

AMP, fructose 2,6 BP

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7
Q

What rxn creates ATP

A

phosphenolpyruvate to pyruvate catalyzed by pyruvate kinase

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8
Q

What inhibits pyruvate kinase

A

ATP and alanine

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9
Q

What induces pyruvate kinase

A

F16BP

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10
Q

What enzyme catalyzes the rxn from pyrvuate to Acetyl-CoA and what inhibits it

A

pyruvate dehydrogenase, ATP, NADH, acetyl-CoA

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11
Q

In which state is FBPase-2 active

A

fasting

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12
Q

In which state is PFK-2 active

A

fed

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13
Q

What regulates whether FBPase-2 or PFK-2 is active

A

protein kinase A

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14
Q

What is the pathway in the fasting state leading to inc FBPase-2 and dec PFK-2

A

inc glucagon, inc cAMP, inc PKA

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15
Q

What is the pathway in the fed state leading to dec FBPase-2 and inc PFK-2

A

inc insulin, dec cAMP, dec PKA

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16
Q

The pyruvate dehydorgenase complex serves in what reaction: reactants

A

pyruvate, NAD+, CoA

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17
Q

The pyruvate dehydrogenase complex serves in what reaction: products

A

Acetly-CoA, CO2, NADH

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18
Q

What co-factors are required for the pyruvated dehydrogenase complex

A

pyrophosphate (B1, thiamine, TPP) FAD (B2, riboflavin), NAD (B3, niacin), CoA (B5 pantothenate), lipoic acid

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19
Q

What activates the pyruvate dehydrogenase complex

A

exercise: inc NAD/NADH, inc ADP, inc Ca

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20
Q

What other complex is similar to the pyruvate dehydrogenase complex in that it has the same co-factors and generates succinyl-CoA

A

alpha-ketoglutarate dehydrogenase complex

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21
Q

What does arsenic do and what are th results of poisoning

A

inhibits lipoic acid, vomiting, rice water stools, garlic breath

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22
Q

What does a pyruvate dehydrogenase deficiency lead to and what are the findings

A

backup of substrate (pyruvate and alanine) resulting in lactic acidosis, congenital or acquired from thiamine def in EtOH, neuro defects

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23
Q

What is the TX for pyruvate dehydrogenase deficiency

A

inc intake of ketogenic nutrients, high in fact content or inc lysine or leucine

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24
Q

What are the only purely ketogenic amino acids

A

Lysine and leucine

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25
Q

Of the four possible fates for pyruvate, which one carries amino groups to liver from muscle

A

alanine

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26
Q

Of the four possible fates for pyruvate, which one can replenish TCA cycle or be used in gluconeogenesis

A

Oxalacetate

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27
Q

Of the four possible fates for pyruvate, which one is a transition from glycolysis to TCA cycle

A

Acetyl-CoA

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28
Q

Of the four possible fates for pyruvate, which ends anaerobic glycolysis as in RBCs, leukocytes, kidney medulla, lens, testes and cornea

A

Lactate

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29
Q

What does the TCA cycle produce per 1 acetyl CoA

A

3 NADH, 1 FADH2, 2 CO2, 1 GTP = 12 ATP (x2 per glucose)

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30
Q

What does Citrate Is Krebs starting substrate for making oxaloacetate

A

citrate, isocitrate, alpha ketoglutarate, succinyl-CoA, succinate, fumarate, malate, oxaleoacetate

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31
Q

How doe NADH electrons from glycolysis and the TCA cycle enter the mitochondiria

A

malate-aspartate shuttle or the glycerol 3 phosphate shuttle

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32
Q

What does the passage of electrons result in that when coupled to OXPHOS drives the production of ATP

A

proton gradient

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33
Q

Where are FADH2 electrons transferred to

A

comlex II

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34
Q

What substances directly inhibit electron transport chain

A

rotenone, CN-, antimycin A, CO

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35
Q

Wgat substances directly inhibit mitochondrial ATPase, causing an inc in proton gradient, no ATP because pump is stopped

A

oligomycin

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36
Q

What are uncoupling agents

A

inc permeability of membrane causing a dec in proton gradient and inc in O2 consumption - ATP synthesis stops, but electron transport continues to produce heat

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37
Q

What substances are uncouling agents

A

2,4 DNP, aspirin

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38
Q

What are the irreversible enzymes of gluconeogenesis

A

pyruvate carboxylase, PEP carboxykinase, fructose 1,6 biphosphatase, glucose 6 phosphatase

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39
Q

Where is pyruvate carboxylase found, what does it do, what does it require amd what activates it

A

in mitochondria, pyruvate to oxaloacetate, requires biotin, ATP, activated by acetyl coA

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40
Q

Where is PEP carboxykinase found, what does it do, and what does it require

A

in cytosol, oxaloacetate to phosphenolpyruvate, requires GTP

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41
Q

Where is fructose 1,6 bisphosphatase found and what does it do

A

cytosol, F 1,6 BP to fructose 6 Phosphate

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42
Q

Where is glucose 6 phosphatase found and what does it do

A

in ER, glucose 6-P to glucose

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43
Q

Where does gluconeogenesis primarily happen and what are other sites where the enzymes are located

A

liver, also in kidney and gut epithelium

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44
Q

What do def in in enzymes of gluconeogenesis cause

A

hypoglycemia

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45
Q

Why can’t muscle produce in gluconeogenesis

A

lacks glucose 6 phophatase

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46
Q

How do odd chain fatty acids participate in gluconeogenesis

A

via 1 proprionyl-CoA which can enter the TCA as succinyl-CoA and undergo gluconeogenesis

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47
Q

Why can’t even chain fatty acids produce new glucose

A

they yield only acetyl-CoA equivalents

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48
Q

What is the purpose of the HMP shunt

A

provide a source of NADPH from an abundantly available glucose 6P, create ribose for nucleotide synthesis and glycolytic intermediates

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49
Q

What are the 2 distinct phases of the HMP shunt and how many ATP are used and produced

A

oxidative and nonoxidative, no ATP produced or used

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50
Q

Which phase of the HMP shunt is reversible and which is irreversible

A

oxidative is irreversible

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51
Q

What happens on the oxidative arm of the HMP shunt and what is the key enzyme

A

glucose 6P to CO2, 2NADPH, ribulose 5P, G6PD, rate limiting step

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52
Q

What happens on the nonoxidative arm of the HMP shunt and what is the key enzyme and cofactor

A

ribulose 5P to ribose 5P, G3P and F6P, transketolase and B1

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53
Q

What does NADPH oxidase deficiency result in and why

A

chronic granulomatous disease, no respiatory burst, no formatino of ROS

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54
Q

What enzyme turns ROS to H2O2

A

superoxide dismutase

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55
Q

What enzyme adds Cl- to the H202 to makes bleach

A

myeloperoxidase

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56
Q

In what cells do the respiratory burst occur

A

PMNs

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57
Q

What substance inside the cell serves to oxidize glutatione

A

peroxide

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58
Q

What substance inside the cell reduces glutatione

A

NADPH

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59
Q

What substance inside the cells replenishes NADPH

A

G6PD

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60
Q

Patients with chronic granulomatous disease are at increased risk of infection from which kind of organisms

A

catalase positive (catalase neg produce H2O2 the cell can use) like S. aureus or aspergillus

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61
Q

What is NADPH’s role inside RBCs

A

keep glutathione reduced so it can detoxify free radicals and peroxides

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62
Q

What does a decrease in decrease in NADPH lead to and why

A

poor defense from oxidizing agents, fava beans, sulfonamides, primaquine, antituberclosis drugs leadig to hemolytic anemia.

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63
Q

What other inflammatory process can induce a hemolytic anemia in NADPH defieicnt patients

A

infection, free radicals generated by inflammatory response

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64
Q

Why is G6PD def more common among patients of african decent

A

protective against malaria

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65
Q

What are Heinz bodies

A

oxidized hemoglobin precipiated within RBCs

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66
Q

What are bite cells and when do you see them

A

result from phagocytic removal of heinz bodies my macs - G6PD def

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67
Q

benign asymptomatic condition with elevated levels of fructose in urine and blood, dz and enzyme

A

essential fructosuria - fructokinase AR

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68
Q

What is the defect in fructose intolerance and what does it cause

A

aldolase B, AR, fructose 1P accumulates causing a dec in availabel phosphate which inhibts glycogenolysis and gluconeogenesis leading to hypoglycemia, cirrhosis, jaundice and vomiting

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69
Q

What enzyme does fructose metabolism bypass to reach glycolysis

A

PFK - rate limiting enzyme

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70
Q

What are possilbe presentation for galactokinase def

A

failure to track objects or develop a social smile

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71
Q

What substance accumulates in galactokinase def and what is the clinical picture

A

galactitol, galactose appears in blood and urine, can cause infantile cataracta - AR

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72
Q

What enzyme results in classic galactosemia and what is the clinical

A

absence of galactose 1 phosphate uridyltransferase, accumulation of toxic substances leads to failure to thrive, jaundice, hepatomegaly, infantile cataracts, mental retardation

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73
Q

In addition to fructose and galactose, what sugards should be excluded from the diets of patients with disorders of fructose or galactose metabolism

A

sucrose = glucose + fructose, lactose = glucose + galactose

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74
Q

What is sorbitol, how and why is it made

A

alcohol version of glucose, can trap glucose in cell, aldose reductase

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75
Q

What sugar is sorbitol converted to and via what enzyme, and what can happen in cells lacking this enzyme

A

fructose via sorbitol dehydrogenase, inc sorbitol leading to osmotic damage as in cataracts, retinopathy, peripheral neuropathy as in diabetes

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76
Q

What tissues have both enzymes of sorbitol metabolism

A

liver, ovaries, seminal vesicles

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77
Q

What tissues have only aldose reductase

A

schwann cells, lens, retina, kidneys

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78
Q

What does lactase deficiency cause

A

loss of brush border enzyme causing bloating, cramps, osmotic diarrhea

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79
Q

Who typically has lactase def

A

African Americans and Asians

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80
Q

What form of amino acids are found in proteins

A

L form

81
Q

What are the glucogenic essential amino acids

A

met, val, arg his

82
Q

What are the glucogenic/ketogenic amino acids

A

Ile, phe, thr, trp

83
Q

What are the purely ketogenic amino acids

A

leu, lys

84
Q

Which are the acidic amino acids

A

Asp and Glu

85
Q

Which are the basic amino acids

A

arg, lys, his, arg is most basic, has has no charge at body pH

86
Q

What two amino acids are required druing periods of growth and why

A

arg and his inc in histones which bind negatively charged DNA

87
Q

What does amino acid catabolsim results in the formation of what

A

common metabolites like pyruvate and acetyl CoA and excess NH4+ converted to urea and exreted

88
Q

What step begins the urea cycle and what is the enzyme needed, where does it happen

A

CO2 + NH4 needs carbamoyl phosphate synthase I, in the mitochondria

89
Q

When does aspartate enter the urea cycle

A

after citruline

90
Q

What is the composition of urea and where do each part derive from

A

NH2-(C=O) -NH2 one NH2 from ammonia the C=O from CO2, and the other NH2 from aspartate

91
Q

How is ammonium transported from muscle to the liver for urea cycle

A

passed to glutamate, then to alanine enters blood, enters liver, coverted to pyruvate and transfers ammonium back to glutamate which gives it to the urea cycle

92
Q

What happens in hyperammonemia

A

depletes alpha-ketoglutarate leading to inhibition of TCA cycle - tremor slurring of speech, somnolence, vomiting, cerebral edema, blurring of vision

93
Q

What is the TX for hyper ammonemia

A

limit protein diet, benzoate or phenylbutarate (both bind amino acids leading to excretion) can decrease ammonia levels

94
Q

What is the most common urea cycle disorder and what is the mode of inheritance?

A

ornithin transcarbamoylase def - x linked recesssive, other urea cycle enzymes defs are autosommal recessive

95
Q

How does OTC def present

A

evident in first few days of life, can present last onest, excess carbamoyl phosphate converted to orotic acid, orotic acid in blood and urine, dec BUN and symptoms of hyerpammonemia

96
Q

What is the amino acid precursor for catecholamines

A

phenylalanine

97
Q

What is the amino acid precuros for niacin and serotonin/melatonin

A

tryptophan

98
Q

What is the amino acid precursor for histamine

A

histidine

99
Q

What is the amino acid precursor for porphyrin and heme

A

glycine

100
Q

What is the amino acid precuror for creatine, urea and nitric oxide

A

arginine

101
Q

What is the amino acid precursor for GABA and glutathione

A

glutamate

102
Q

What enzyme converts phenylalanine to tyrosin

A

phenylalanine hydroxylase

103
Q

What converts tyrosine to DOPA

A

tryosine hydroxylase

104
Q

What converts DOPA to dopamine

A

dopa decarboxylase

105
Q

What converts dopamine to NE

A

dopamine beta hydroxylase

106
Q

What converts NE to epi

A

phenylethamolamine N methyl transferase

107
Q

What is the breakdown product of dopamine

A

HVA

108
Q

What is the breakdown product of NE

A

VMA

109
Q

What is the breakdown product of epi

A

metanephrine

110
Q

decreases in what substances can cause PKU

A

phenylalanine hydroxylase, tetrahydrobiopterin cofactor

111
Q

What enzyme becomes essential in PKU

A

tyrosine

112
Q

What does inc phenylalanine lead to

A

phenylketones in urine

113
Q

What are the findings in PKU

A

mental retardation, growth retardation, seizures, fair skin, eczema, musty body odor

114
Q

What is the TX for PKU

A

dec phenylalanine (contained in aspartame, Nutrasweet) inc tyrosine in diet

115
Q

What is maternal PKU

A

lack of proper dietary therapy during pregnancy leading to microcephaly, mental retardation growth retardation, congenital heart defects

116
Q

What creates the musty body odor in PKU

A

disorder of aromatic amino acid metabolism

117
Q

congenital deficiency of homogentisic acid oxidase in the degradative pathway of tyrosine to fumarate leading to dark connective tissue, brown pigmented sclera, urine turns black on standing - dz and worst complication

A

alkaptonuria, may have debiliating arthralgias

118
Q

What are the two possible causes of albinism

A

lack of tyrosinase (no melanin) AR ordefective tyrosine transporters (less tyrosine) - can results in lack of migration of NC cells

119
Q

Pts with albinism are at inc risk for what cancer

A

skin

120
Q

Why is albinism inheritnace varialbe due to

A

locus heterogeneity - ocular albinism is x-linked recessive

121
Q

What are the 3 AR forms of homocystinuria

A

cystathionine synthase def, dec affinity of cystathionine synthase for pyridoxal phosphate, homocystein methyltransferase def

122
Q

What is the treatment for cystathionine synthase def

A

dec methionine, inc cystiene, inc B12/folate

123
Q

What is the treatment for dec affinity of cystathionine synthase for pyroxidal phosphate

A

inc vit B6

124
Q

What are the findings with homocystinuria and what amino acid is needs to be supplemented

A

homocystein in ruine, mental retardation, osteoporosis, tall stature, kyphosis, lens subluxation and atherosclerosis leading to stroke and MI, cysteine

125
Q

What is the defect in cystinuria

A

hereditary defect of renal tubular amino acid transporter for cystein, ornithine, lysine and arginine in PCT

126
Q

What is the complication of cystinuria

A

cystine kidney stones, cystine staghorn calculi - cystine is made of two cysteines connected by disulfide bond

127
Q

What causes maple syrup urine disease and what does it lead to

A

dec in alpha ketoacid dehydrogenas leading to blocked degredation of branches amino acids like Ile, Leu, and Val - severe CNS defects mental retardation and death

128
Q

What causes Hartnup’s disease

A

AR disorder characterized by defective neutral amino acid transporte on renal and intestinal epith cells

129
Q

What does hartnups disease cause

A

tryptophan excretion in urine and dec absorption from the gut leading to pellagra

130
Q

How do glucagon/epi lead to glycogenolysis

A

Adenylyl cycle, inc cAMP, inc PKA, glycogen phosphorylase kinase activated, glycogen phosphoylase active, glycolysis

131
Q

How does insulin inhibit glycogenolysis

A

receptor tyrosine kinases, protein phosphatase, takes phosphate off glycogen phosphorylase kinase inactivating it

132
Q

What else can phosphoylate phosphorylase kinase

A

Ca/calmodulin in muscle to coordinate with muscle activity

133
Q

What kind of branches do glycogen branches have

A

alpha 1,6 and alpha 1,4

134
Q

What happens do glycogen in skeletal muscle during exercise

A

glycogenolysis to form glucose

135
Q

What happens to glycogen in the liver

A

stored and undergoes glycogenolysis to maintain blood sugar at appropriate levels

136
Q

What enzyme converts glucose 1 p to UDP glucose

A

UDP glucose pyrophosphorylase

137
Q

What enzyme degrades a small amount of glycogen in lysosomes

A

alpha 1,4 glucosidase

138
Q

What liberates glucose from glucose 6 P

A

glucose 6 phosphatase

139
Q

What converts limit dextran to glucose

A

debranching enzyme

140
Q

Very Poor Carbohydrate Metabolism stands for 4 of the glycogen storage diseases, what are thy

A

Von Gierke’s, Pompe, Cori, McArdle

141
Q

Severe fasting hypoglycemia, inc glycogen storage in liver, inc blood lactate, hepatomegaly - dz and def enzyme

A

von gierkes, glucose 6 phosphatase

142
Q

cardiomegaly, systemic findings leading to early death - dz and enzyme

A

pompe’s lysosomal alpha 1,4 glucosidase (acid maltase) (pompe trashes the pump; heart, liver, muscle)

143
Q

Milder form of type I with nl blood lactate levels - dz and enzyme

A

cori’s, debranching enzyme alpha 1,6 glucosidase, gluconeogenesis intact

144
Q

inc glycogen in muscle but can’t break it down, painful muscle cramps, myglobinuria with strenuous exercise - dz and enzyme

A

mcardle’s, skeletal muscle glycogen posphorylase

145
Q

peripheral neuropathy of hands/feet, angiokeratomas, CV/renal disease - dz, def enzyme, acc substrate, inherit

A

fabrys, alpha galactosidase A, ceramide trihexoside, XR

146
Q

hepatosplenomegaly, aseptic necrosis of femur, bone crisis, MACS that look like crumpled tissue paper

A

gaucher’s, beta glucocerebrosidase, glucocerebrosie, AR

147
Q

progressive neurodegeneration, hepatosplenomegaly, cherry red spot on macula, foam cells

A

neimann-pick, sphingomyelinase, sphingomyelin, AR

148
Q

progressive neurodegeneration, developmental delay, cherry red spot on macula, lysosomes with onion skin, NO hepatosplenomegaly

A

Tay-Sachs, hexosaminidase A, GM2 ganglioside, AR

149
Q

peripheral neuropathy, developmental delay, optic atrophy, glopoid cells

A

krabbes, galactocerebrosidase, galactocerebroside, AR

150
Q

central and peripheral demyelination with ataxia and dementia

A

metachromatic leukodystrophy, arylsulfatase A, cerebroside sulfate AR

151
Q

developmental delay, gargoylism, airway obstruction, corneal clouding, hepatosplenomegaly

A

hurlers syndrome, alpha L iduronidase, heparan sulfate/dermatan sulfate, AR

152
Q

Mild Hurlurs + aggressive behavior no corneal clouding

A

Hunters, iduronate sulfatase, heparan sulfate/dermatan sulfate, XR

153
Q

What shuttle is used in fatty acid degredation and what does it move and from where to where

A

carnitine shuttle, acyl-coa from cyto to mito

154
Q

What inhibits the carnitine shuttle

A

malonyl coa

155
Q

What happens in carnitine def

A

inability to transport LCFAs into mitochondria results in toxic accumulation causing weakness, hypotonia, hyperketotic hyperglycemia

156
Q

What shuttle is involved in fatty acid synthesis and what does it move from where to where

A

citrate, acetyl coa from mito to cyto

157
Q

What does beta oxidation do and where does it occur

A

breaks down acyl-coa to acetyl coa groups in mito

158
Q

what happens in acyl coa dehyrdogenase def

A

inc dicarboxylic acids, dec in glucose and ketones

159
Q

What does acetyl-CoA become before becoming palmitate

A

malonyl coa (+ biotin= palmitiate, 1 16C fatty acid)

160
Q

What enzymes metabolize fatty acids and amino acids

A

acetoacetate and beta hydroxybutyrate

161
Q

What occurs to oxaloacetate in starvation and DKA

A

depleted for gluconeogenesis staling the TCA cycle and shunting glucose and FFA to production of ketone bodies

162
Q

What happens to oxaloacetate in alcholism

A

excess NADH shunts oxaloacetate to malate (backwards) stalling the TCA and shunting glucose and FFA to ketone body production

163
Q

What are ketone bodies made from, where are they metabolized and how are they excreted

A

HMG-CoA, brain to 2 molecules of acetyl-Coa, excreted in urine

164
Q

in a 100 meter sprint where does energy come from

A

stored ATP, creatine phosphate, anaerobic glycolysis

165
Q

In a 1000 meter run, where does energy come from

A

same as sprint + OXPHOS

166
Q

in a marathon where does energy come from

A

glycogen and FFA oxidation; glucose conserved for final sprinting

167
Q

What are the priorities for the body in fasting and starvation

A

supply sufficient glucose to brain and RBCs and to preserve protein

168
Q

What is the energy source in the fed state right after a meal

A

glycolysis and aerobic respiration

169
Q

What is the source of energy in the fasting state between meals

A

hepatic glycogenolysis&raquo_space; hepatic gluconeogenesis > adipose release of FFA

170
Q

What are the blood glucose levels maintained by for days 1-3

A

hepatic glycogenolysis, adipose tissue release of FFA, muslce and liver FFA, hepatic gluconeogenesis from peripheral tissue lactate and alanine and from adipose tissue glycerol and proprionyl-coA (odd chain FFA)

171
Q

What is the energy source after day 3 of starvation

A

adipse tissue stores, keton bodies become the main source of energy fo the brain and heart, after these are depleted, vital protein degradation accelerates, leading to organ failure and death

172
Q

When are glycogen reserves depleted

A

after day 1

173
Q

1 g of protein or cabrohydrate = ?kcal

A

four

174
Q

I g fat = ? Kcal

A

nine

175
Q

What is the rate limiting enzyme in cholesterol synthesis

A

HMG-CoA (HMG-CoA to mevalonate

176
Q

What enzyme esterifies 2/3 of plasma cholesterol

A

LCAT (lecithin cholesterol acyltransferase)

177
Q

What does lipoprotein lipase do

A

degredation of TG circulating in chylomicrons and VLDLs

178
Q

What does pancreatic lipase do

A

degredation of dietary TG in small intestine

179
Q

What does hepatic TG lipase do

A

degradation of TG remaining in IDL

180
Q

What does hormone sensitive lipase do

A

degradation of TG stored in adipocytes

181
Q

What CETP do

A

transfers cholesterol from mature HDL to VLDL, IDL and LDL (cholesterol ester transfer protein)

182
Q

What does apoA 1 do

A

activates LCAT

183
Q

What does apoB100 do

A

binds to LDL receptor, mediates VLDL secretion

184
Q

What does apoCII do

A

cofactor for LPL

185
Q

What does apoB48 do

A

mediates chylomicron secretion

186
Q

What does apoE do

A

mediates extra remnant take up

187
Q

Delivers dietary TGs to peripheral tissues, delivers cholesterol to liver in the form of remnants, mostly depleted of TGs, secreted by intestinal epith cells

A

chylomicrons

188
Q

What apolipoproteins are on chylomicrons

A

b48, AIV, CII, E

189
Q

delivers hepatic TGs to peripheral tissue, secreted by liver

A

VLDL

190
Q

What apolipoprotiens are on VLDL

A

B-100, CII and E

191
Q

Formed and degradation of VLDL, delivers TGs and cholesterol to liver where they are degraded to LDL

A

IDL

192
Q

what apolipoproteins are on IDL

A

B100 and E

193
Q

Delivers hepatic cholesterol to peripheral tissues, formed by LPL modification of VLDL in the peripheral tissue, taken up by target cells via RME

A

LDL

194
Q

What apolipoprotein is on LDL

A

B100

195
Q

Mediates reverse cholesterol transport from periphery to liver, acts as respository for apoC an apoE, secreted from both liver and small intestine

A

HDL

196
Q

What is the defect in I- hyperchylomicronemia

A

LPL def, or altered apoCII, elevated TG and cholesterol, causes pancreatitis, hepatosplenomegaly and eruptive/pruritic xannthomas - no risk of atherosclerosis

197
Q

What is the defect in II A familial hypercholesterolemia

A

AD absent of dec LDL receptors causes accelerated atherosclerosis, achilles xanthomas and corneal arcus, increase LDL and elecated cholesterol

198
Q

what is the defectin IV - hypertriglyceridemia

A

hepatic overproduction of VLDL causing pancreatitis, elvelated TGs and VLDL

199
Q

How does abetalipoproteinemia present and what is the defect

A

early in life, AR, inability to synthesize lipoproteins due to def in apoB100 and B48 - intestinal biopsy shows accumulation within enterocytes due to inability to export absorbed lipid as chylomicrons - failure to thrive, steatorrhea, acanthocytosis, ataxia and night blindness

First Aid Biochem (3 decks)

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