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FA 2015 High Yields > Biochemistry High Yields > Flashcards

Biochemistry High Yields Flashcards

1
Q

mRNA stop codons

A

UGA, UAA, UAG
“U Go Away”
“U Are Away”
“U Are Gone”

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2
Q

Rough Endoplasmic Reticulum

A

Site of synthesis of secretory proteins and of N-linked oligosaccharide addition to many proteins
Goblet cells of small intestines and plasma cells are rich in RER

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3
Q

Smooth Endoplasmic Reticulum

A

Site of steroid synthesis and detoxification of drugs and poisons; Lacks surface ribosomes
Liver hepatocytes and adrenal cortex and gonads are rish in SER

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4
Q

Golgi

A

Distribution center for proteins and lipids from ER to the vesicles and plasma membrane; Modifies N-oligosaccharide on aspargine; Adds O-oligosaccharides on serine and threonine; Adds mannose-6-phosphate to proteins for trafficking to lysosomes

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5
Q

I-Cell Disease

A

Inherited lysosomal storage disorder; defect in N-acetylglucosaminyl-1-phosphotransferase –> failure of golgi to phosphorylate mannose residues (ie. decreased mannose-6-phosphate) on glycoproteins –> proteins secreted extracellularly rather than delivered to lysosomes
Coarse facial features (gargoyl), clouded corneas, restricted joint movement, high plasma levels of lysosomal enzymes, increased acid hydrolases and glycosylases, hyperplastic gums

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6
Q

Drugs that Act on Microtubules

A

Mebendazole, Griseofulvin, Colchicine, Vincristine/Vinblastine, Paclitaxel

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7
Q

Type I Collagen

A

Bone, skin, tendon, cornea; Osteogenesis Imperfecta

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8
Q

Type II Collagen

A

Cartilage

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9
Q

Type III Collagen

A

Reticulin - skin, blood vessels, uterus, granulation tissue; Vascular type of Ehlers-Danlos

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10
Q

Type IV Collagen

A

Basement membrane, basal lamina, lens; Alport syndrome, Goodpasture syndrome

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11
Q

Gq

A

H1, Alpha1, V1, M1, M3 - “HAVe 1 M&M”; Phospholipase C –> IP3/DAG –> Ca2+/PKC

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12
Q

Gs

A

Beta1, Beta2, D1, H2, V2; Adenylyl cyclase –> cAMP, PKA

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13
Q

Gi

A

M2, Alpha2, D2 - “MAD 2s”; decrease cAMP

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14
Q

Dynein

A

Retrograde to microtubule (+ –> -)

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15
Q

Kinesin

A

Anterograde to microtubule (- –> +)

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16
Q

GFAP Stain

A

Neuroglial cells

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17
Q

Collagen Synthesis

A

1) Synthesis (RER) - Translation of collagen alpha chains
2) Hydroxylation (RER) - hydroxylation of specific proline and lysine residues (requires Vit. C)
3) Glycosylation (RER) - formation of procollagen via hydrogen and sulfide bonds (triple helix)
4) Exocytosis into extracellular space
5) Proteolytic processing - cleavage of disulfide rich terminal regions –> insoluble tropocollagen
6) Cross-linking - to make collagen fibrils
* Glycine is predominant AA along with lysine and proline

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18
Q

Ehlers Danlos

A

Problem with cross-linking of collagen

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19
Q

Southern Blot

A

DNA with DNA probe

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20
Q

Northern Blot

A

RNA with DNA probe; useful for studying mRNA levels which are reflective of gene expression

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21
Q

Western Blot

A

Protein with antibody probe

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22
Q

Southwestern Blot

A

Identifies DNA-binding proteins using labeled oligonucleotide probes

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23
Q

X-Linked Recessive Disorders

A

Bruton agammaglobulinemia; Wiskott-Aldrich syndrome; Fabry disease; G6PD deficiency; Ocular albinism; Lesch-Nyhan; Duchenne; Hemophilia, Ornithine transcarbamylase deficiency

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24
Q

Trinucleotide Repeat Expansion Diseases

A

Fragile X - CGG
Friedreich Ataxia - GAA
Huntington - CAG
Myotonic Dystrophy - CTG

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25
Q

Chr. 3

A

von Hippel Lindau, Renal cell carcinoma

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26
Q

Chr. 4

A

ADPKD with PKD2 defect; Huntington disease

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27
Q

Chr. 5

A

Cri-du-chat syndrome, FAP

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28
Q

Chr. 7

A

Williams syndrome; CF

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29
Q

Chr. 9

A

Friedreich Ataxia

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30
Q

Chr. 11

A

Wilms Tumor

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31
Q

Chr. 13

A

Patau syndrome, Wilson Disease

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32
Q

Chr. 15

A

Prader Willi, Angelman Syndrome

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33
Q

Chr. 16

A

ADPKD with PKD1 defect

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34
Q

Chr. 17

A

NF-1

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35
Q

Chr. 18

A

Edwards Syndrome

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36
Q

Chr. 21

A

Down Syndrome

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37
Q

Chr. 22

A

NF-2; DiGeorge syndrome

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38
Q

Fat Soluble Vitamins

A

A, D, E, K; Malabsorption with CF or Celiac

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39
Q

Vit B1 (Thiamine) Reactions

A

Pyruvate Dehydrogenase (links glycolysis to TCA)
Alpha-Ketoglutarate Dehydrogenase (TCA cycle)
Transketolase (HMP Shunt)
Branched Chain Ketoacid Dehydrogenase

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40
Q

Vit B2 (Riboflavin)

A

Component of flavins FAD and FMN used in redox rxns.

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41
Q

Vit. B3 (Niacin)

A

Constituent of NAD+ and NADP+; Derived from tryptophan

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42
Q

Vit. B5 (Pantothenic Acid)

A

Essential component of coenzyme A and fatty acid synthase

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43
Q

Vit B6 (Pyridoxine)

A

Converted to pyridoxal phosphate, a cofactor in transamination, decarboxylation and glyocogen phosphorylase;
Synthesis of cystathionine, heme, niacin, histamine, 5-HT, Epi, NE, Dopamine and GABA
*Isoniazide therapy/OCPs can lead to peripheral neuropathy, convulsions etc.

44
Q

Vit B7 (Biotin)

A

Cofactor for carboxylation enzymes:

  • Pyruvate carboxylase: pyruvate –> oxaloacetate (gluconeogenesis)
  • Acetyl-CoA carboxylase: acetyl CoA –> malonyl CoA (FA synthesis)
  • Propionyl-CoA carboxylase: propionyl-CoA –> methylmalonyl CoA (Odd carbon FA synthesis)
45
Q

Vit B9 (Folate)

A

Converted to THF, a coenzyme for 1-carbon transfer/methylation reactions
Deficiency can be caused by: phenytoin, sulfonamides, methotrexate
Labs in deficiency: increased homocystein and normal methylmalonic acid

46
Q

Phosphofructokinase-1

A

Glycolysis

AMP +, F-2,6-bisphosphate +, ATP - , citrate -

47
Q

Fructose-1,6-bisphosphate

A

Gluconeogenesis

ATP +, Acetyl-CoA +, AMP -, F-2,6-bisphosphate -

48
Q

Isocitrate Dehydrogenase

A

TCA Cycle

ADP +, ATP -, NADH -

49
Q

Glycogen Synthase

A

Glycogenesis

G-6-P +, Insulin +, cortisol +, Epi -, Glucagon -

50
Q

Glycogen Phosphorylase

A

Glycogenolysis

Epi +, Glucagon +, AMP +, G-6-P -, Insulin -, ATP -

51
Q

Glucose-6-Phosphate Dehydrogenase (G6PD)

A

HMP Shunt

NADP+, NADPH -

52
Q

Carbamoyl Phosphate Synthetase II

A

De Novo Pyrimidine Synthesis

ATP+, UTP-

53
Q

Acetyl-CoA Carboxylase (ACC)

A

Fatty Acid Synthesis

Insulin +, Citrate +, Glucagon -, Palmitoyl-CoA -

54
Q

Glutamine-Phosophoribosylpyrophosphate Amidotransferase

A

De Novo Purine Synthesis

AMP-, Inosine Monophosphate-, GMP-

55
Q

Carbamoyl Phosphate Synthetase I

A

Urea Cycle

N-acetylglutamate+

56
Q

Carnitine Acyltransferase I

A

Fatty Acid Oxidation

Malonyl-CoA-

57
Q

HMG-CoA Synthase

A

Ketogenesis

58
Q

HMG-CoA Reductase

A

Cholesterol Synthesis

Insulin +, Thyroxine +, Glucagon -, Cholesterol -

59
Q

Pyruvate Carboxylase

A

Pyruvate –> Oxaloacetate
Requires biotin and ATP; Activated by Acetyl-CoA
In mitochondria

60
Q

Phosphoenolpyruvate Carboxykinase

A

Oxaloacetate –> Phosphoenolpyruvate
Requires GTP
In cytosol

61
Q

Fructose-1,6-Bisphosphatase

A

F-1,6-bisphosphate –> F-6-P
Citrate+, F-2,6,-bisphosphate -
In cytosol

62
Q

G-6-P

A

G-6-P–> glucose

In ER

63
Q

Essential Fructosuria

A

Defect in fructokinase

Symptoms: asymptomatic except fructose appears in blood and urine

64
Q

Fructose Intolerance

A

Defect in Aldolase B; F-1-phosphate accumulates –> decrease in available phosphate –> inhibition of glycogenolysis and gluconeogenesis
Symptoms present after consumption of fruit, juice or honey
Urine dipstick -; reducing sugar can be detected in urine
Symptoms: hypoglycemia, jaundice, cirrhosis, vomiting
Tx: decrease intake of fructose and sucrose

65
Q

Galactokinase Deficiency

A

Deficiency of galactokinase; Galactitol accumulates if galactose is present in diet
Symptoms: galactose appears in blood and urine; infantile cataracts; may present as failure to track objects or to develop a social smile

66
Q

Classic Galactosemia

A

Absence of galactose-1-phosphate uridyltransferase;
Damage caused by accumulation of toxic substances (including galactitol, which accumulates in the lens of the eye)
Symptoms: failure to thrive, jaundice, hepatomegaly, infantile cataracts, intellectual disability
Tx: exclude galactose and lactose

67
Q

Essential AAs

A

Glucogenic: Methionine, Valine, Histidine
Glucogenic/Ketogenic: Isoleucine, Phenylalanine, Threonine, Tryptophan
Ketogenic: Leucine, Lysine

68
Q

Ornithine Transcarbamylase Deficiency

A

X-linked recessive; Interferes with body’s ability to eliminate ammonia; Excess carbmoyl phosphate is converted to orotic acid
Findings: increased orotic acid in blood and urine, decreased BUN, symptoms of hyperammonemia; No megaloblastic anemia (unlike Orotic aciduria)

69
Q

PKU

A

Decreased phenylalanine hydroxylase or tetrahydrobiopterine cofactor
Increased phenylalanine –> excess phenylketonse in urine
Findings: intellectual disability, growth retardation, seizures, fair skin, eczema, musty body odor
Tx: decrease phenylalanine and increase tyrosine in diet

70
Q

Maple Syrup Urine Disease

A

Blocked degradation of branched amino acids (Isoleucine, Leucine, Valine) due to decreased alpha-ketoacid dehydrogenase; Causes increased alpha-ketoacids in the blood (esp. leucine)
Causes severe CNS defects, intellectual disability and death
Tx. Thiamine supplementation and restriction of branched AAs
“I Love Vermont Maple Syrup from trees with branches”

71
Q

Homocystinuria

A

Types:
- cystathionine synthase deficiency
- decreased affinity of cystathionine synthase for pyridoxal phosphate
- homocysteine methyltransferase deficiency
Findings: increased homocysteine in urine, intellectual disability, osteoporosis, marfanoid habitus, kyphosis, lens subluxation, thrombosis and atherosclerosis (stroke and MI)

72
Q

Glycogen Breakdown Enzymes

A

1) UDP-glucose pyrophosphorylase
2) Glycogen synthase
3) Branching enzyme
4) Glycogen phosphorylase
5) Debranching enzyme (4-alpha-D-glucanotransferase)
6) Debranching enzyme (alpha-1,6-glucosidase)

73
Q

Orotic Aciduria

A

Inability to convert orotic acid to UMP (de novo pyrimidine synthesis pathway) because of defect in UMP synthase;
Presents as failure to thrive, developmental delay and megaloblastic anemia refractory to folate or B12; No hyperammonemia (unlike ornithine transcarbamylase deficiency); Orotic acid in urine
Tx: uridine monophosphate to bypass mutated enzyme

74
Q

Von Gierke Disease

A

G-6-phosphatase deficiency
Findings: hepatomegaly, hypoglycemia, severely increased glycogen in liver, increased blood lactate (lactic acidosis), increased triglycerides, increased uric acid (gout), short stature, doll-like facies, protruding abdomen, hepatic steatosis
Tx. Frequent oral glucose/cornstarch; avoidance of fructose and galactose

75
Q

Pompe Disease

A

Lysosomal alpha-1,4-glucosidase (acid maltase) deficiency leading to increased glycogen accumulation
Findings: cardiomegaly, hypertrophic cardiomyopathy, exercise intolerance, NO hepatomegaly, NO hypoglycemia, early death
“Pompe trashes the pump”

76
Q

Cori Disease

A 
Debranching enzyme (alpha-1,6-glucosidase) deficiency
Milder form of Von Gierke (hypoglycemia, hypertriglyceremia, ketoacidosis, hepatomegaly) with normal blood lactate levels; Accumulation of abnormally short outer dextrin-like structures in cytosol of hepatocytes without histopathological fatty infiltration of liver; Can also effect muscles
Gluconeogenesis is intact
77
Q

McArdle Disease

A

Skeletal muscle glycogen phosphorylase (myophosphorylase) deficiency
Findings: Increase glycogen in muscle, but cannot be broken down –> painful muscle cramps, myoglobinuria (red urine) with strenuous exercise and arrythmia from electrolyte abnormalities
Blood glucose levels typically normal
Tx. with vit B6

78
Q

Fabry Disease

A

Alpha-galactosidase A deficiency leading to accumulation of Ceramide trihexoside (XR)
Findings: Peripheral neuropathy of hands/feet, angiokeratomas, cardiovascular/renal disease

79
Q

Gaucher Disease

A

Glucocerebrosidase (beta-glucosidase) deficiency leading to accumulation of Glucocerebroside (AR)
Findings: hepatosplenomegaly, pancytopenia, osteoporosis, aseptic necrosis of femur, bone crises, Gaucher cells (lipid–laden macrophages resembling tissue paper)
Tx. Recombinant glucocerebrosidase

80
Q

Niemann-Pick Disease

A

Sphingomyelinase deficiency leading to accumulation of Sphingomyelin (AR)
Findings: Progressive neurodegeneration, hepatosplenomegaly, foam cells (lipid-laden macrophages), cherry red spot on macula (retinal pallor sparing macula), sphingomyelinase in histiocytes (sea blue), Zebra bodies (lamellar lipid deposits)
More common in Ashkenazi Jews

81
Q

Tay-Sachs Disease

A

Hexosaminidase A deficiency leading to accumulation of GM2 ganglioside (AR)
Findings: progressive neurodegeneration, developmental delay, cherry red spot on macula, lysosomes with onion skin, NO hepatosplenomegaly, blindness

82
Q

Krabbe Disease

A

Galactocerebrosidase deficiency leading to accumulation of glactocerebroside, psychosine (AR)
Findings: peripheral neuropathy, developmental delays, optic atrophy, globoid cells

83
Q

Metachromatic Leukodystrophy

A

Arylsulfatase A deficiency leading to accumulation of Cerebroside sulfate (AR)
Findings: Central and peripheral demyelination with ataxia and dementia

84
Q

Hurler Syndrome

A

Alpha-L-iduronidase deficiency leading to accumulation of Heparan sulfate, dermatan sulfate
Findings: developmental delay, gargoylism, airway obstruction, corneal clouding, hepatosplenomegaly

85
Q

Hunter Syndrome

A

Iduronate sulfatase deficiency leading to accumulation of Heparan sulfate, dermatan sulfate
Findings: mild Hurler + aggressive behavior, NO corneal clouding, kyphosis, short stature, vascular and ischemic heart disease

86
Q

Systemic Primary Carnitine Deficiency

A

Defect in transport of LCFAs into mitochondria –> toxic accumulation
Findings: weakness, hypotonia, hypoketotic hypoglycemia
Decreased acetoacetate and beta-hydroxyburyrate

87
Q

Medium-Chain Acyl-CoA Dehydrogenase Deficiency

A

AR disorder of fatty acid oxidation; Decreased ability to break down fatty acids into acetyl-CoA –> accumulation of 8-10 Carbon fatty acyl carnitines in blood and hypoketotic hypoglycemia
Presents: in infancy or early childhood with vomiting, lethargy, seizures, coma and liver dysfunction; can lead to sudden death

88
Q

Ketone Urine Test

A

Does not detect Beta-hydroxybutyrate

89
Q

Apolipoprotein E

A

Mediates remnant uptake;

Chylomicron, chylomicron remnant, VLDL, IDL, HDL

90
Q

Apolipoprotein A-I

A 
Activates LCAT (cholesterol esterification)
Chylomicron, HDL
91
Q

Apolipoprotein C-II

A

Lipoprotein lipase cofactor

Chylomicron, VLDL, HDL

92
Q

Apolipoprotein B-48

A

Mediates chylomicron secretion

Chylomicron, chylomicron remnant

93
Q

Apolipoprotein B-100

A

Binds LDL receptor

VLDL, IDL, LDL

94
Q

Chylomicron

A

Delivers dietary TG to peripheral tissue; Delivers cholesterol to liver in form of remnants, which are mostly depleted of their TGs; Secreted by intestinal epithelial cells

95
Q

VLDL

A

Delivers hepatic TG to peripheral tissue; secreted by liver

96
Q

IDL

A

Formed in the degradation of VLDL; delivers TG and cholesterol to liver

97
Q

LDL

A

Delivers hepatic cholesterol to peripheral tissues; Formed by hepatic lipase modification of IDL in peripheral tissue. Taken up by target cells via receptor-mediated endocytosis

98
Q

HDL

A

Mediates reverse cholesterol transport from periphery to liver; acts as repository for Apolipoproteins C and E (needed for chylomicron and VLDL metabolism); Secreted by both liver and intestines; Alcohol increases synthesis

99
Q

Hyper-Chylomicronemia

A

Increased Chylomicrons, TG, cholesterol in blood
Lipoprotein lipase deficiency or altered apolipoprotein C-II
Causes pancreatitis, hepatosplenomegaly, erruptive/pruritic xanthomas; No increased risk of atherosclerosis; Creamy layer in supernatant

100
Q

Familial Hypercholesterolemia

A

Increased LDL, cholesterol
Absent of defective LDL receptors
Causes accelerated atherosclerosis, tendon xanthomas, corneal arcus

101
Q

Hypertriglyceridemia

A

Increased VLDL, TG
Hepatic overproduction of VLDL
Hypertriglyceridemia, acute pancreatitis

102
Q

RNA Pol I

A

rRNA (ricin poisoning effects this)

103
Q

RNA Pol II

A

mRNA (amatoxins from mushrooms effect this)

104
Q

RNA Pol III

A

tRNA

105
Q

Sickle Cell Mutation

A

Valine for glutamic acid

FA 2015 High Yields (10 decks)

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