Special Topics Flashcards
1
Q
GAGs are made of alternating
A
Acidic sugar (glucuronic acid/iduronic acid) Amino sugar (n-acetylglucosamine/galactosamine)
2
Q
All GAG are covalently attached to proteins to form proteoglycans except
A
Hyaluronic acid
3
Q
GAG linked to proteins via?
A
Trihexoside serine-o-glycosidic bonds
4
Q
GAGs
With structural role in sclera
A
Dermatan sulfate
5
Q
GAGs
Role in corneal transparency
A
Keratan sulfate and dermatan sulfate
7
Q
Glycoproteins: Major classes (3)
A
O linked
N linked
GPI linked
8
Q
Glycoproteins:
Bound to serine and threonine
Golgi
Hydroxyl group
A
O-linked
9
Q
Glycoproteins:
Bound to asparagine
Amide group
ER
A
N-linked
10
Q
Glycoproteins:
O-linked in fertilization
A
ZP3
11
Q
Deficient phosphorylation of mannose residues in N-linked glycoprotein enzymes –> no labelling of pre-enzymes so that they can be transported to lysosomes
Presents with coarse facial features, skeletal abnormalities, severe psychomotor impairment
A
I-cell disease
12
Q
Glycoproteins:
In influenza virus, these allows new viruses to exit the cell
A
Neuraminidase
13
Q
Glycoproteins:
HIV type 1:
Used to attach to cell?
Used to fuse with the host?
A
GP 120
GP 41
14
Q
Glycoproteins:
Disease associated with an altercation in the glycosylation of IgG; no galactose in Fc; ends in GlcNAc; IgM attacks IgG
A
Rheumatoid arthritis
15
Q
Attachment is mediated by a GPI present on the surface of its parasite
A
Malaria
16
Q
GAG found in synovial fluid, vitreous humor, and loose connective tissue
A
Hyaluronic acid
17
Q
Sugars in Hyaluronic acid
A
GlcNAc, GlcUA
18
Q
GAG found in cartilage, bone and cornea
A
Chondroitin sulfate
19
Q
Sugars found in Chondroitin sulfate
A
GalNAc, GlcUA
20
Q
GAG in cornea and loose connective tissue
A
Keratan sulfate
21
Q
Sugars in Keratan sulfate
A
GlcNAc, Gal
22
Q
GAG in mast cells
A
Heparin
23
Q
Sugars in Heparin
A
GlcN, IduA
24
Q
GAG in skin and aortic wall
A
Heparan sulfate
25
Q
Sugars in Heparan sulfate
A
GlcN, GlcUA
26
GAG found in wide distribution
Dermatan sulfate
27
Sugars in dermatan sulfate
GalNAc, IdUA
28
MPS Disease:
Deficiency and findings:
Alpha l iduronase
Corneal clouding
Type I H
| Hurler's
29
MPS Disease:
Deficiency and findings:
Alpha l iduronase
Cardiomyopathy
Mental retardation
Type I S
| Scheie's
30
MPS Disease:
Findings:
Spasticity then loss of motor function
Mental retardation
Hyperactivity
Type III
| San Filippo syndrome
31
4 enzymes in San filippo syndrome?
Heparan sulfamidase
N-acetylglucosaminidase
N-acetylglucosamine sulfatase
N-acetylglucosamine
32
MPS Disease:
Deficiency and findings:
Iduronate sulfatase
NO corneal clouding
Cardiomyopathy
Mental retardation
Type II
| Hunter syndrome
33
MPS Disease:
Deficiency and findings:
Galactose 6 sulfatase
No CNS involevement
Skeletal dysplasia and short stature
Type IV
Morquio's syndrome
34
MPS Disease:
Deficiency and findings:
B-glucuronidase
Corneal clouding
Hepatomegaly
Skeletal dysplasia and short stature
Mental retardation
Type VII
| Sly syndrome
35
Glycoprotein vs proteoglycan
| Which is longer, linear and with repeating sugar units?
Proteoglycans
36
Glycoprotein vs proteoglycan
| Which is shorter, branched, with no repeating sugar?
Glycoprotein
37
Average daily nutrient intake level estimated to meet the requirement of 50% of healthy individuals in a particular life stage and gender group
Estimated average requirement
38
Average daily dietary intake level that is sufficient to meet the requirements of 95% of all individuals in a life stage and gender group
Recommended dietary allowance
39
Set in the absence of scientific evidence to compute for EAR or RDA
Adequate intake
40
Highest average nutrient intake level to pose no risk of adverse health effects
Tolerated upper intake level
41
Estimated energy requirement:
Sedentary?
Moderately active?
Very active?
30-35-40 kcal/kg/day
42
Energy requirement:
Fat?
CHO?
Protein?
Energy requirement:
Fat? 20-35%
CHO? 45-65%
Protein? 10-35%
43
Energy content:
Fats?
CHO/Proteins/Ketones?
Alcohol?
Energy content:
Fats? 9 kcal/g
CHO/Proteins/Ketones? 4 kcal/g
Alcohol? 7 kcal/g
44
How is energy used?
BMR?
Diet induced thermogenesis?
Exercise?
How energy is used?
BMR? 60%
Diet induced thermogenesis? 10%
Exercise? 30%
45
```
Kwashiorkor vs Marasmus
>1 year old
Stunting
With edema
Very low plasma protein
With fatty liver, skin lesions and depigmented hair
```
Kwashiorkor
46
```
Kwashiorkor vs Marasmus
<1 year old
Stunting and extreme wasting
Without edema
Normal plasma protein
With anemia
```
Marasmus
47
Vitamin A
| 3 forms?
Retinoic acid
Retinol
Retinol
48
```
Vitamin A
Functions?
Retinoic acid
Retinol
Retinol
```
Retinoic acid - epithelial growth regulators
Retinol - gametogenesis in gonads
Retinol - rod and cone cells for vision
49
Vitamin A
Storage form?
Transport form?
Vitamin A
Storage form? Retinyl ester
Transport form? Retinol binding protein
50
Vitamin D
| 3 forms
Ergocalciferol (D2) - plant, milk
Cholecalciferol (D3) -animal, skin
Calcitriol
51
Vitamin D
Precursor?
Storage form?
Transport form?
Vitamin D
Precursor? 7 dehydrocholesterol
Storage form? 25 OH D3
Transport form? 1,25 OH D3
52
PTH vs Vitamin D:
Increases PO4
Increases intestinal absorption of calcium
Vitamin D
53
PTH vs Vitamin D:
Decreases PO4
Indirectly Increases intestinal absorption of calcium
PTH
54
Vitamin Deficiency in:
RBC fragility
Neurologic dysfunction
Vitamin E
55
```
Vitamin toxicity in:
Hyperkeratosis
Hepatomegaly
Pseudotumor cerebri
Fractures
Teratogenicity
```
Vitamin A
56
Vitamin K
| 3 forms
Phylloquinone (K1)
Menaquinone (K2)
Menadione (Synthetic)
57
Vitamin K toxicity?
Jaundice + hemolytic anemia
58
Vitamin K deficiency?
Rare
| Hemorrhagic disease of the newborn
59
Water soluble vitamins generally nontoxic except:
B6
| B12
60
Glycosphingolipids in ABO system?
A: N-acetylgalactosamine
B: Galactose
AB:Both
61
Cofactor in:
Proline and lysine hydroxylation (collagen synthesis)
Dopamine B hydroxyoase (dopa to norepi)
Fe3+ to Fe2+
Vit C
62
Biotin Carboxylation reactions
Pyruvate - gluconeogenesis
Acetyl coa - FA synthase
Propionyl coa - FA
63
Vitamin Def:
Avidin in eggwhites
Dermatitis
Enteritis
Biotin
64
Vit Def:
Megaloboastic anemia with no neuro symptoms
Neural tube defects
Folic acid (B9)
65
Folic acid (B9) supplementation in pregnant mothers
400 mcg daily
66
Folic acid structure:
Pterin ring + PABA + glutamate residues
67
Folic acid:
| Active form:
THF
68
Folic acid:
Enzymes:
PABA to DHF
DHF to THF
Dihydropteroate synthase
| Dihydrofolate reductase
69
```
Vitamin functions as:
Coenzyme for 1-carbon transfer
Methylation reaction (purine and thymine synthesis)
```
Folic acid (B9)
70
Vitamin B12 active forms:
For methionine synthesis?
For isomerization reactions?
5-deoxyadenosylcobalamin
| Methylcobalamin
71
```
Vitamin deficiency in:
Pernicious anemia
Neuropsychiatric sx
Schillin test as Dx
Anti IF as Dx
```
Cobalamin
72
Active form of B6
Pyridoxal phosphate
73
Vitamin as coenzyme for:
```
AA transamination
Glycogen phosphorylase
Cystathione synthase
ALA synthase
Niacin synthesis from tryptophan
```
Pyridoxine (B6)
74
Vit def in:
| Isoniazid toxicity
Pyridoxine
75
Pyridoxine toxicity
2g/day
76
Vitamin as constituent of Coenzyme A
| Acyl transfer & FA synthase
Panthothenic acid
77
Vit def sx:
Dermatitis
Enteritis
Alopecia
Panthothenate
78
Riboflavin active forms
FMN
| FAD
79
Vitamin as cofactors in redox reactions as electron carrier
Riboflavin
80
```
Deficiency symptoms:
Stomatitis
Cheilosis
Dermatitis
Corneal revascularization
```
Riboflavin
81
Niacin active forms
NAD
| NADP
82
Vitamin as coenzymes in redox reactions, as electron carriers
Niacin
83
Derived from tryptophan using B6
Niacin
84
Niacin deficiency
```
Pellagra
Hartnup (dec tryptophan absorption)
Carcinoid syndrome (inc tryptophan metabolism)
Isoniazid use (dec vit b6)
```
85
Cofactor in pyruvate, alpha ketoglutarate and branched chain AA dehydrogenase
Cofactor in transketolase reactions in the HMP shunt
Thiamine
86
Dry vs wet beriberi:
| No heart failure, polyneuritis, symmetrical muscle wasting
Dry
87
Wet beriberi
High output cardiac failure
88
Grams of iron in the body
3-4 g
89
2/3 of iron in:
Hemoglobin
90
Grams of zinc
1.5 to 2.5 g
91
```
Constituent of:
Carbonic anhydrase
Cytoplasmic superoxide dismutase
Alcohol dehydrogenase
Carboxypeptidases
DNA and RNA polymerase
```
Zinc!
92
Diet: meats, nuts, beans, wheat germ
Zinc!
93
Dermatitis, diarrhea, alopecia secondary to zinc malabsorption
Acrodermatitis enteropathica
94
Grams of copper
80-110 g
95
```
Enzyme cofactor:
Cytochrome oxidase
Dopamine b hydroxylase
Monoamine oxidase
Tyrosinase
Delta 9 desaturase
Lysyl oxidase
Cytoplasmic superoxide dismutase
```
COPPER!
96
```
Microcytic hypochromic anemia
Leukopenia
Hemorrhagic vascular changes
Bone demineralization
Hypercholesterolemia
Neurologic problems
```
COPPER!
97
TPN and infants
| Vit def?
COPPER!
98
X linked recessive
Deficiency of ATP dependent membrane transporter for copper
Growth retardation, mental deficiency, seizures, arterial aneurysm, bone demineralization, brittle hair
Menkes Syndrome
99
Hepatolenticular degeneration
| Biliary excretion of copper is blocked, with accumulation at liver and brain
Wilson Disease
100
Found in Wilson disease
Kayser fleischer rings
101
Tx for Wilson disease
D-penicillamine
102
Trace element which causes madness (psychosis, parkinsonism)
Manganese
103
Occurs in few oxidase enzymes like xanthine oxidase
Molybdenum
104
Def causes cardiomyopathy (endemic in china)
Selenium (Kashan disease)
105
Trace element in glutathione peroxidase
Selenium
106
Xenobiotics
| Phase 1
Hydroxylation
107
Xenobiotics
| Phase 2
Conjugation
