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1
Q

glycogen supplements contain

A
  • macro glycogenic nutrients
  • micro intracellular ergogenic substrates
  • most body can use to restore: 0.7 g/kg
  • ‘recommended’ - 76 g
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2
Q

glycogen phosphorylase converts from

A

n residues –> n-1 residues + glucose-1-phosphate

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3
Q

phosphorylation of GP causes

A
  • the amino terminus of the protein (res 10-22) to swing through 120 degrees, moving into the subunit interface and moving Ser-14 by more than 3.6 nm (CONFORMATIONAL CHANGE)
  • 9 ser residues on GP are phosphorylated
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4
Q

for glycogen synthesis, how many residues are phosphorylated?

A

5 Ser residues on GS are phosphorylated

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5
Q

normal liver size for adult and 10 yo

A

adult liver: 2.2 kg (4.8 lbs)

10 yo liver: 1.2 kg (2.5 lbs) — becomes up to 7 lbs (10% bpdy weight in Von Gierke’s)

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6
Q

molecular defects in Glucose-6-phosphatase deficiency (and location)

A

(Von Gierkes)
Type 1a: absence of activity of catalytic subunit of glucose-6-Pase enzyme complex
Type 1b: glucose-6-Pase transport
Type 1c: microsomal phosphate or pyrophosphate transport
Type 1d: microsomal glucose transport

Syria and Lebanon- serious form of disease
Saudi Arabia- mild

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7
Q

Glucose-6-P can go to

A
  • PPP –> synthesis of NADPH and 5-C sugars
  • Fructose-6-P –> the rest of glycolysis
  • Glucose-1-P –> Glycogen (energy storage in liver/muscle) or Glucuronate
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8
Q

The side products of PPP go to

A

the 2 NADPH go to reductive anabolic pathways (fatty acid synthesis)

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9
Q

how is NAD+ converted to NADP+?

A

via NAD+ kinase

only difference between NAD+ and NADP+ structurally is a PO4- added where one of the OH’s used to be

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10
Q

XK in PPP

A

may play an important role in metabolic disease, given that Xu5P is a key regulator of glucose metabolism and lipogenesis

D-xylulose –> xylulose-5-P via XK

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11
Q

important products/purposes of PPP

A

oxidative phase: NADPH to fatty acid synthesis

non-oxidative: ribose-5-P to nucleic acid biosynthesis (DNA/RNA), Fructose-6-P and glyceraldehyde-5-P as glycolytic intermediates

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12
Q

target for cancer therapy

A
  • NADK
  • Due to the essential role of NADPH in lipid and DNA biosynthesis and the hyper proliferative nature of most cancers, NADK is an attractive target for cancer therapy

Also:
- phosphatidic acid may act as a target model for novel anti-cancer drugs

  • Nucleotide synthesis pathways are good targets for anti-cancer/antibacterial strategies

Gln PRPP amidotransferase:

  • Site of action for Azaserine
  • Antibiotic and anti-tumor agent
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13
Q

phosphatidylcholine

A

a complex membrane lipid

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14
Q

size of glycogenin

A

37 kDA homodimer

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15
Q

visceral fat linked to

A

metabolic disturbances and increased risk of CVD and T2D

  • in women, breast cancer, the need for gallbladder surgery
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16
Q

excess fat stored in

A

lipocytes (which expand size until fat is used for fuel)

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17
Q

values of energy per unit weight of different energy sources:

A

fat: 37 kJ
protein: 17 kJ
glycogen: 16 kJ
glucose: 16 kJ

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18
Q

mass of different energy sources

A

fat: 15,000g (83%) of stored energy
protein: 6,000g
glycogen: 190g
glucose: 20g

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19
Q

essential fat% for men and women

A

men: 2-4%
women: 10-12%

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20
Q

acceptable fat % for women/men + obese fat%

A

acceptable:

  • 25-31% for women
  • 18-25% for men

obese:
- 32+% for women
- 25%+ for men

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21
Q

what do pancreatic lipases do?

A

break triacylglycerols down into diacylglycerols and monoacylglycerols

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22
Q

lipase enzymes

A

a diverse array of genetically-distinct lipase enzymes are found in nature and represent several types of protein folds and catalytic mechanisms
- most are built on an alpha/beta hydrolase fold and employ a mechanism using a catalytic triad consisting of a serine nucleophile, a histidine base, and an acid residue (usually Asp)

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23
Q

mixed micelles are made up of

A

long-chain fatty acids + bile salts

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24
Q

Knoop concluded that

A

phenyl products (phenyl acetate and benzoate) could only result from beta-oxidation if carbons were removed in pairs

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25
what are the important side products of beta-oxidation and when are they produced?
NADH (produced in step that uses hydroxyacyl-CoA DH) FADH2 (produced in step that uses acyl-coA DH)
26
reactions in beta-oxidation and where we've seen it before
oxidation -> hydration -> oxidation (first three) (same as TCA) note: fourth is reverse Claisen condensation that cleaves the beta-keto ester
27
reaction 1 of beta oxidation
fatty acyl-CoA --> trans-delta enoyl-CoA enzyme: Acyl-CoA dehydrogenase produces FADH2 from FAD
28
where does Acetyl-CoA come from (before entering Krebs)
breakdown of proteins, polysaccharides and FATS (to FAs)
29
connection between ETS + beta oxidation
FADH2 enters flavoprotein 3 which goes to UQ/UQH2 pool
30
ATP yield from beta oxidation for palmitic acid (C16)
- 8 acetyl-CoAs - 7 NADH - 7 FADH2 (106 ATP)
31
Beta-oxidation - oxidizing odd-carbon FA's: the steps
propionyl coA -> D-Methylmalonyl-CoA --> L-Methylmalonyl-CoA --> Succinyl-CoA Enzymes: (1) Propionyl-CoA carboxylase (2) Methylmalonyl-CoA epimerase (D-> L conversion) (3) Methylmalonyl-CoA mutase (rearrangement)
32
For beta-oxidation -- oxidizing odd carbon FAs, what are two coenzymes involved and which enzymes are they involved with?
biotin + B12 Biotin: propionyl-CoA carboxylase is biotin-dependent (the first enzyme that converts propionyl-CoA) B12: Methylmalonyl-CoA mutase (the enzyme that converts to succinyl-CoA)
33
What is the enzyme number of Enoyl-CoA isomerase and what does it do?
EC 5.3.3.8 converts cis to trans-delta acyl CoA
34
Path of beta-oxidation for unsaturated FAs
three cycles of beta oxidation -> cis-delta-dodeconyl-CoA -------(ECI)-----> trans-delta-dodecenoyl-CoA ---> (now normal beta-oxidation) trans-delta-dodecenoyl-CoA -> beta-hydroxyacyl-CoA (via enoylCoA hydratase).....
35
Alcohol Dehydrogenase reactions
Ethanol (CH3CH2OH) ----> Acetaldehyde (CH3CHO) via ADH Acetaldehyde ---> Acetic acid (CH3COOH) via ALDH --> excreted OR Acetaldehyde --> GSH-adduct (via glutathione) Acetaldehyde is TOXIC
36
Acute Cerebellar Ataxia Symptoms:
- impaired coordination in the torso or arms and legs - frequent stumbling - unsteady gait - uncontrolled or repetitive eye movements - trouble eating and performing fine motor tasks - slurred speech - vocal changes - headaches
37
name one cis, one trans, and one saturated fat
oleic: cis elaidic: trans stearic: saturated
38
reaction of beta-oxidation (for palmitic acid)
CH3(CH2)14CO-CoA + 108 Pi + 23 O2 --> 108 ATP + 16 CO2 + 130 H2) + CoA
39
three ketone bodies
acetoacetate, acetone, beta-hydroxybutyrate
40
ketogenesis pathway
acetyl-CoA ----------(beta-ketothiolase)------> acetoacetyl-CoA -----(HMG-CoA synthase)-----> HMG-CoA --------(HMG-CoA lyase)-------> acetoacetate -----> acetone and beta-hydroxybutyrate (the second one is via b-hydroxybutyrate DH)
41
HMG-CoA connection between paths
HMG-CoA is part of formation of ketone bodies from acetyl-CoA and also forms cholesterols via HMG-CoA reductase
42
FA synthesis pattern and how it relates to other processes we've seen
reduction- dehydration-reduction (opposite of beta-oxidation)
43
what coenzyme is involved in acetyl-CoA carboxylase reaction?
BIOTIN (because ACC uses bicarbonate, ATP)
44
Animal ACC enzyme
one polypeptide with all three functions: biotin carboxyl carrier, biotin carboxylase, transcacrboxylase (E. coli enzyme has three separate units)
45
Bacteria/Plants: Domain 1 enzymes
Domain 1: - beta-ketoacyl-ACP synthase - malonyl transferase enzymes are separate and in a complex
46
FA Synthase in animals: what happens in each domain?
FA synthase is a multi enzyme complex Domain 1: acyl and malonyl binding and condensation Domain 2: reduction of domain 1 intermediate Domain 3: liberation of palmitate product
47
How does lipogenesis work?
- Excess glucose from the blood enters adipocyte --> TAG - insulin - VLDL and chylomicrons enter adipocytes --> fatty acids --> TAG
48
glucagon is associated with
low blood glucose
49
biosynthesis of glycogen
extracellular glucose -> intracellular glucose -> glucose-6-P -> glucose-1-P -> UDP-glucose -> short chain of glucose polymer --------> glycogen
50
Lecithin/Sphingo (L/S) ratio for baby development
L:S ratio: mature: >1.9 (PG present) borderline: 1.5-1.9 (PG absent) immature: <1.5 (PG absent) mature (diabetic mothers): >3.4
51
Spiders
- Brown recluse venom, is cytotoxic/hemolytic. - It contains hyaluronidase, deoxyribonuclease, ribonuclease, alkaline phosphatase, and lipase and sphingomyelinase D. - Sphingomyelinase D responsible for most of the tissue destruction and hemolysis. - The intense inflammatory response mediated by arachidonic acid, prostaglandins, and chemotactic infiltration of neutrophils
52
production of prostaglandins, thromboxjnes, prostacyclin
membrane phospholipids -> arachidonic acid arachidonic acid --> prostaglandins, thromboxjnes (this is inhibited by NSAIDS) arachidonic acid --> prostaglandins, prostacyclin (inhibited by NSAIDS, COX-2 inhibitors)
53
tetrahedral intermediate is key for
HMG-CoA reductase (in cholesterol biosynthesis)
54
pregnenolone is formed where? progesterone is formed where?
pregnenolone: formed in mitochondria progesterone: formed in ERA
55
diseases associated with estrogen
- heart disease - cancer (1/8 women, 85% survival in 5 years) - stroke - more
56
sources of omega 3, 6, 9
omega 3: essential FA - DHA, EPA: fish - ALA: flax seeds, chia seeds omega 6: essential FA - grape seed oil, sesame oil omega 9: non-essential FA - olive oil, canola oil
57
ki for mevinolinic acid (related to statins)
ki = 0.6 nM
58
side effects of colesevelam hydrochloride (cholestagel)
- constipation - abdominal pain - bloating - vomiting - diarrhea - weight loss - flatulence
59
lipoprotein density
highest density: HDL (also most protein compared to lipids) | lowers density: chylomicrons, VLDL
60
molecular weight of apolipoproteins
Apo A-1: 29,000 Apo B-100: 513,000 Apo C-II: 8800 Apo E: 34,000
61
LDL + LDL receptor interaction - key apoproteins
Apo B-100 and ApoE key in this interaction | - B-100 is embedded in surface of the LDL receptor
62
transaminations (for purine synthesis) - dependent on what coenzyme
pyrudixal phosphate
63
IMP Biosynthesis: Reaction 1
Enzyme: Ribose-5-P Pyrophosphokinase | - Ribose-5-P --> 5-Phosphoribosyl-pyrophosphate
64
IMP Biosynthesis: Reaction 2
Enzyme: Glutamine PRPP amidotransferase 5-PRPP --> Phosphorybosyl-beta-amine
65
Folic acid reduces risk of
cancer (colon, breast cancer), senility/Alzheimer's
66
What is unique about Alcohol Dehydrogenase?
its Zn site (structural stability role)
67
name of the drug that inhibits ALDH
antabuse
68
what's a process NADH is used for
fatty acid synthesis
69
what is inosine-5-P?
a purine nucleotide
70
Importance of DPA, EPA, DHA
- DPA is important factor for preventing CVD - EPA is a huge factor in fighting inflammation - DHA is an essential molecule for brain, nerve and eye tissues, and is a powerful factor for normalizing blood and tissue triglycerides
71
What does LP lipase do and what is it activated by?
LP lipase is activated by apoC-11. LP lipase hydrolyzes TAGs
72
Source of Nitrogen in plants and microorganisms
- In these organisms, glutamate is the source of N, via transamination (aminotransferase) reactions

Biochem Test 2 (7 decks)

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