Biochemistry Test 2

Front 1

Uses of Glucose

Back 1

1) ATP via glycolysis
2) storage as glycogen
3) glycoproteins
4) pentoses → generate NADPH

Front 2

Glycolysis

Back 2

can be aerobic / anaerobic

anaerobic: glyc. only energy source

Front 3

Glycolytic Pathway

Back 3

Glc.+ADP+2Pi→2 Lactate+2ATP+2H2O

Front 4

[Lactate] >>> [lactic acid] @ phys. pH

Back 4

use of "lactate" more correct -- largely unprotonated

Front 5

Erythrocyte ATP source

Back 5

glycolysis only

Front 6

Cardiac Muscle & Brain ATP source

Back 6

ox. metabolism (all cond)

Front 7

Skeletal Muscle ATP source

Back 7

ox. metabolism (at rest)

both ox. metabolism & glyc. (exercise)

Front 8

Hemolytic Anemia

Back 8

loss of fxn of glycolytic enzymes

(hits RBCs especially hard due to lack of mito)
*can affect skel. muscle more during exertion

Front 9

NAD+

Back 9

Nicotinamide Adenine Dinucleotide

derived from B-vitamin niacin

Front 10

Negative Cooperativity

Back 10

binds multiple molecules, but each subsequent addition is more difficult than the preceeding one

Front 11

E req. to convert soluble protein from random coil to folded?

Back 11

from an increase in entropy of water when hydrophobic residues become buried

Front 12

Methemoglobinemia

Back 12

↑ levels Methemoglobin in blood (HbM)

HbM does not bind O2

can be caused by nitrates and nitrites (Acq.)

inherited from diaphorase deficiency, or Pyr Kin def. --> ↓ NADH

shortness of breath, cyanosis, mental status changes, headache, fatigue, exercise intolerance, dizziness and loss of consciousness

Front 13

Trypsin

Back 13

- pocket, thus binds specifically + substrates

Front 14

Elastase

Back 14

Narrow pocket, binds small substrates

Front 15

HPV E6

Back 15

targets p53

Front 16

HPV E7

Back 16

targets pRB, releases E2F

Front 17

PFK1

Back 17

1st committed step

inhib. by excess ATP, citrate, FA

activated F2,6BP

Front 18

Wernike-Korsakoff Syndrome

Back 18

ataxia, mental disturb., uncoordinated eye mvmts.

stems from thiamine deficiency, responds to suplementation

Front 19

I-cell disease

Back 19

inclusions in lysosomes

phosphorylation defect prevents trafficking of enz to lys.

Mannose-6-Phosphate is Lysosome "zip-code"

Front 20

Glucose 6 Phosphate Dehydrogenase deficiency

Back 20

G6PDH catalyzes NADPH formation

↑ sensitivity to oxidative damage (esp RBC -- Hb precipitate, inflexibility, damage)

triggered by drugs such as antibiotics or primaquines

Front 21

Galactosemia

Back 21

failure to thrive, acidosis, jaundice, retardation, cataracts, elevated [galactose] in blood/urine

accumulate gal-1-p (gal not harmful)

galactose-1-phos uridyltransferase def.

Front 22

Maltose

Back 22

Glc - Glc alpha 1-4

Front 23

Lactose

Back 23

Galactose - Glucose beta 1-4

Front 24

Sucrose

Back 24

Glucose - Fructose alpha 1-2

Front 25

Amylose

Back 25

linear glc. polymer alpha 1-4

Front 26

alpha linkage

Back 26

O below plane

Front 27

beta linkage

Back 27

O above plane

Front 28

Mucopolysaccharidoses

Back 28

failure to degrade glucopolysaccharides

↓ fxn enz --> accum. partially degraded material

Front 29

Heparin

Back 29

anti-coagulant

binds antithrombin II, inhibits

Front 30

Raffinose sugars

Back 30

from beans, humans lack enzyme to break down

Front 31

Lactase Intolerance

Back 31

common Africain / Asian, worsens w/ age

can be caused by disease damage to enterocytes

avoid lactose & take lactase in pill form

Front 32

MODY

Back 32

mature onset diabetes of the young

autosomal dom. glucokinase mut.

Front 33

Hemolytic Anemia

Back 33

loss of fxn in glycolytic enzyme

most common: Pyr Kinase def. (affects RBC and working skeletal musc.)

Front 34

Pellagra

Back 34

poor growth / weight loss, skin rash, diarrhea, mental disturbances

stems from niacin deficiency (nec. for NAD+ form)

Front 35

Irreversible Glycolytic Steps

Back 35

hexokinase
PFK
Pyr Kinase

Front 36

Hereditary Fructosuria

Back 36

fructokinase def.

no significant clinical issues

Front 37

Hereditary Fructose Intolerance

Back 37

aldolase B def.

accum. F-1-P

sweating, trembling, dizziness, nausea, vomiting, convulsions, coma

Front 38

Thiamine (vitamin B1)

Back 38

from cereals, grains, pork, legumes, seeds, nuts

def. leads to Beri-Beri, edema, weight loss, CHF, Wernicke's encepalopathy, confusion, irritability

Front 39

Leigh's Disease

Back 39

def. Pyr Dehydrogenase

neurological effects, loss of head control, motor skills, seizures, lactic acidosis

treat w/ thiamine or vit. B1

prognosis: poor

Front 40

Type I GSD: Von Dierke Diease

Back 40

G6P.ase -

liver, kidney, intestine

severe fasting hypoglycemia, no response to epi, glucagon
lactic acidosis, growth retardation, renal disease

Front 41

Type II GSD: Pompe Disease

Back 41

lysosomal a1-4 glucosidase -

broad effects, liver, heart, musc.

accumulate normal glycogen in cytoplasmic vacuoles; norm. blood glc.

Front 42

Type III GSD: Cori's Disease

Back 42

Debranching -

can't use glyc. stores, still do Glc.neo.genesis

Front 43

Type IV GSD: Andersen's Disease

Back 43

Branching -

long, linear glyc., large heart/spleen

*catastrophic

Front 44

Type V GSD: McArdle Disease

Back 44

skeletal musc. glyc. phosphorylase -

adult onset, exercise difficult
normal glyc., but hard to access

Front 45

Type VI GSD: Hers Disease

Back 45

Liver Glyc. Phosphorylase -

hepatomegaly
no response to glucagon

Front 46

Type VII GSD: Tauri's Disease

Back 46

PFK-

can't do glycolysis, fructose helps

Glycogen normal in structure

Front 47

GSD Mnemonic

Back 47

Viagara Pills Cause A Major Hardon Tonight
Go Lay Down Before My Libido Peaks

V: Von Gierk Disease, Type I, Glucose 6-phosphatase deficiency

P: Pompe Disease, Type II, lysosomal alpha1-4 glucosidase deficiency

C: Cori's Disease, Type III, Debranching enzyme deficiency

A: Andersen's Disease, Type IV, Branching enzyme deficiency

M: McArdle Disease, Type V, Muscle glycogen phosphorylase deficiency

H: Her's Disease, Type VI, Liver glycogen phosphorylase deficiency

T: Touri's Disease, Type VII, PFK I deficiency in muscle