B1U3- Imp Stuff2

Front 1

name 3 Microcytic Anemias (3)
-req in which enzyme or
something special

Back 1

1). Vit 6 def:
-req in ALA synthase

2). Iron def:
**hypochromic** (pale RBC)

3). Lead poisoning:
-ferrochelase inhib
-ringed sideroblasts in bm (dec ferritin-inc Fe in mit)

Front 2

What causes inc indirect bilirubin in bld?

Back 2

inc indirect bilirubin = unconjugated
-hemolysis (G6PD, pyruvate kinase, vit K tox, hemolytic anemia)
-Crigler-Najier/Gilberts Syndrome
-newborn jaundice (inc RBC dest & UDP-glucuronyl transferase induced at birth)
-hepatic damage (Wilson's, Hep

Hint 2

heme synthesis & ded

Front 3

kernicterus

Back 3

inc bilirubin (unconjugated/indirect)

Hint 3

heme synthesis & ded

Front 4

What causes inc direct bilirubin in the bd??

Back 4

direct bilirubin= conjugated
-anything wrong w/ bile duct (stone/cancer)
-hepatic damage (Wilson's, Hep)
-Dubin-Johnson (drk pigm in liver)
-Rotor Syndrome

Hint 4

heme synthesis & ded

Front 5

Colors:
biliverdin
bilirubin
urinary bilirubin
stercobilins
urobilins

Back 5

Colors:
biliverdin- green
bilirubin- orange
urinary bilirubin (pathological)- brown urine (conjugated)
stercobilins- brown = poop
urobilins- yellow (non pathological pee)

Hint 5

heme synthesis & ded

Front 6

ALA synthase:
-what it does
-cofactor required
-how it is regulated (2)

Back 6

ALA (delta-aminolevulinic acid) synthase
-B6
-succinyl-CoA + gly --> ALA

ALA1- hepatic
(-) heme
(+) phenobarbital

ALA2-erythroid
(-) low Fe... (IRE 5`)... translation inhibited

Hint 6

heme

Front 7

1st porphyrin of pathway

Back 7

UPPgIII (uroporphryinogen III)

Hint 7

heme

Front 8

vit K
-food source
-what kills it (2)
-vit K dep proteins

Back 8

vit K
-green leafy veg
-antibiotics kill gut fluora & warfarin
-vit k cep: 2, 7 , 9, 10, C & S

Hint 8

bleed

Front 9

factors w/ short half lives

Back 9

7, C, S

Hint 9

bleed

Front 10

warfarin pt: they will ___ (bleed or clot) bc C and S proteins will be destroyed long before prothrombin (factor 2) will be destroyed

Back 10

CLOT

Hint 10

bleed

Front 11

Hemophilia is more common in ___
VW disease is more common in ___

Back 11

hemophilia is more common in men (women are carriers)
VW disease occurs EQUALLY is men and women

Hint 11

bleed

Front 12

Rather have platelet prob or deep clot prob??

Back 12

rather have platelet prob (always better to bleed than to clot)

Hint 12

bleed

Front 13

PLATELET/BV disorders
-two broad categories

Back 13

If bleeding is immediate....
1).muco:
-nose,mouth,GI tract,menstration,gums

2).cutaneous(skin)
-superficial-you can see bruise
-peticiae (red dots on ankles)
-blood bullae on oral mucosa

Hint 13

bleed

Front 14

COAGULATION disorders
-two terms

Back 14

-bruises you feel, not see
-deep in muscle:
**hematomas = blood lump (in brain for example)
**hemarthrosis = bleeding into joints

Hint 14

bleed

Front 15

blood count tells you (2)

Back 15

# of platelets
size of platelets
(young = big & sticky)

Hint 15

bleed

Front 16

prothrombin time

Back 16

-extrinsic & common

Hint 16

bleed

Front 17

activated partial thromboplastin time

Back 17

-intrinsic & common

Hint 17

bleed

Front 18

thrombin clotting time of fibrinogen

Back 18

time it takes fibrinogen to convert to fibrin
** tells if you have enough fibrinogen
**tells if fibrinogen/fibrin is working well

Hint 18

bleed

Front 19

whole blood platelet function screening

Back 19

-tells if platelets are working right
(tell nothing about vascular function or bv)

Hint 19

bleed

Front 20

mature RBC lack what?
-only do what pathways?

Back 20

mature RBC lack nuc, mit, organelles
- ANAEROBIC glycolysis & PPP

Hint 20

RBC

Front 21

HIF-1
-what is it? where produced?
-stimulated by what? stimulates what?
-influences what?

Back 21

intracellular TF produced in kidneys
-stimulated by apoxia (low O2)
-influences EPO to stimulate RBC production

Hint 21

RBC

Front 22

2,3-BPG production
(when & under what condit is it produced)
-in lungs vs muscles
**what enzymes are involved??

Back 22

LUNGS (basic): G3P dehydrogenase binds phosphoglycerate kinase --> anaerobic glycolysis
-2,3-BPG not made (O2 binds in R state)

MUSCLES
-G3D binds phophomutase and produces 2,3-BPG, which stabilizes T state (O2 released)

Hint 22

RBC

Front 23

3 functions of reduced glutathion (GSH)

Back 23

-detox of hydrogen peroxidase
-reduction of oxidized protein thiols
-reduction of met-hemoglobin

Hint 23

RBC

Front 24

Cyanosis

Back 24

heme is oxidized from Fe2+ to Fe3+
-Fe3+ cannot bind O2

Hint 24

RBC

Front 25

low O2 levels and 2,3 BPG synthesis

Back 25

low O2 levels stimulates 2,3 BPG synthesis

Hint 25

RBC

Front 26

Peripheral Tissue vs Lung HCO3-/Cl- and RBC

Back 26

Peripheral Tissue:
low O2, high CO2,
RBC exports more HCO3- (Cl- comes in)

Lungs:
low CO2, high O2,
RBC takes in HCO3- (exports Cl-)

Hint 26

RBC

Front 27

ABO bld group ag
A locus --> encodes for
H locus --> encodes for
B locus --> encodes for

Back 27

A locus --> GalNaC transferase
H locus --> fucosyl transferase
B locus --> Gal transferase

(AB = universal donor, O = univ acceptor)

Hint 27

RBC

Front 28

most iron in abs in the ____
fats are digested in the _____ and abs in the _____

Back 28

iron abs in the duodenum

fat digested in duogenum & abs in the ileum

Hint 28

heme

Front 29

infection and iron absorption
**how is this stimulated??**

Back 29

during infection iron abs dec

-infection stimulates HEPCIDIN,
which causes FERROPORTIN dedregation

Hint 29

heme

Front 30

most absorbed Fe source to least

Back 30

most = heme
Fe2+
least = Fe3+

Hint 30

heme

Front 31

inadequate production of
stomach acid and iron uptake (2)

Back 31

-promotes Fe3+ (dec abs)
-also DMT2 transport doesn't work

Hint 31

heme

Front 32

conditions that enhance uptake of iron
from enterocytes to bld (6)

Back 32

-inc EPO
-hypoxia
-hemolysis
-hemorrhage
-low iron stores
-ANEMIA

Hint 32

heme

Front 33

disease of too little iron (1)
diseases of too much iron (3)

Back 33

disease of too little iron (1)
-anemia of chronic disease

diseases of too much iron (3)
-classic hereditary hemochromatosis
-acruloplasminemia
-beta-thalassemia

Hint 33

heme

Front 34

iron deficiency anemia:
3 stages

Back 34

iron deficiency anemia:
1). dec serum ferritin
-no sign of storage
2). -less iron bound to transferrin
(less iron is being transported
-more transferrin receptors

3). hypochromic erythrocytes
(less iron available = pale color)

Hint 34

heme

Front 35

vit B12
-name
-2 enzymes it is involved w/
-deficiency: name + 2 consequences

Back 35

vit b12 = cobalamin
1). methylmalonyl-CoA isomerase
2). methionine synthase
(homocystine +CH2-THF --> met + THF)

**pernicious anemia = vit B12 def
(dec erythropoiesis, deg of spinal cord)

Hint 35

heme

Front 36

uroporphyrin vs coproporphyrin structurally (p. 532)

Back 36

UPP: propionyl & acetyl
CPPP: methyl & propionyl

*Ass press report on europe
*Military press cooperate

Hint 36

heme

Front 37

Microcytic Anemia Vit 6 def:
-req in which enzyme?
-ALA synthase inc/dec
-inc/dec protophyoria
-inc/dec ferritin
-ringed sideroblasts in bm??
-serum iron: inc or dec??
-caused by what??

Back 37

Vit 6 def:
- req in ALA synthase
-protophyoria: dec
-ferritin: inc
-ringed sideroblasts in bm (inc Fe in mit)
-ALA: dec
-serum iron: inc
-cause: TB drugs (isoniazid)

Front 38

Microcytic Anemia: Iron def

-ALA synthase inc/dec
-inc/dec protophyoria
-inc/dec ferritin
-ringed sideroblasts in bm??
-serum iron: inc or dec??

Back 38

**hypochromic** (pale RBC)
-protophyoria: inc
-dec ferritin
-ALA-norm
-serum iron: dec

Front 39

Microcytic Anemia: Lead poisoning:
-req in which enzyme?
-ALA synthase inc/dec
-inc/dec protophyoria
-inc/dec ferritin
-ringed sideroblasts in bm??
-serum iron: inc or dec??

Back 39

3). Lead poisoning:
-ferrochelase inhib
-protophyoria: inc
-ringed sideroblasts in bm (dec ferritin-inc Fe in mit)
-ALA-inc
-serum iron: dec

Front 40

describe the apical and basolateral aa transporters
& ass DISEASE:

1).Na+ dep aa (apical)
2).Cationic aa (apical)
3).Na+ dep neutral aa (apical)
4).Na+ dep anionic aa (apical)
5).Cationic aa (basolateral)

Back 40

1).Na+ uptake of Gly and Pro.
AMINO GLYCINURIA
2).ornithine, lys&Arg (Na+ idep)
CYSTINURIA
3) Na+ dep neutral aa;
activity w/ gly & pro is limited.
HARTNUP DISORDER
4).Na+ dep transp Asp & Gln
DICARBOXYLIC AMINOACIDURIA
5). basolat tranp
LAT1- neutral for cationic
LYSINURGIC protein intol
lys,arg,ornithine transp in sml intest & kid
LAT2- exchanges neutral aa for neutral aa

Hint 40

aa met 335

Front 41

difference bet LAT1 & LAT2
tranp

Back 41

LAT1- exchanges cationic aa for neural aa ***LYSINURGIC PROTEIN INTOLERANCE (lys, arg, ornithine)

LAT2- exchanges neurtral aa for neutral aa

Front 42

what is anion gap? how is it calculated?

Back 42

with K+:serum concentration of cholride and bicarbonate (anions) from the concentrations of sodium plus potassium (cations) = ([Na+]+[K+]) - ([Cl-] + [HCO3-])

w/o K+:[Na+] - ([Cl-] + [HCO3-])

***anion gap reflects anions not measured like albumin**
**ORGANIC ACIDURIAS CONTRIBUTE TO ANION GAP***

Front 43

Isovaleric acidemia: what is the prosthetic group of the defective enzyme?

Back 43

Isovaleryl-CoA dehydrogenase
*flavin dep (FAD -->FADH2 --> NADH)

Front 44

Isovaleric academia:
treatment: what is is & reason behind it

Back 44

Isovaleric academia :
-dec dietary protein (urea cycle messed up)
-oral GLYCINE & intravenous CARNITINE
-isovaleric acid & 3-hydroxyvaleric acid accumulate
-rxn = isovaleryl-carnitine & isovalerly-glycine

Front 45

ACE2 and Collectrin transporters
&disease involved
(two types of the disease related to these)

Back 45

**neutral aa transporters; Na+ dep
ACE2: stimulates transp activity
(vasodilation/hypotension)

Collectrin: memb protein involved in trafficking

HARTNUP DISORDER (dec Try)
1). Kidney form: tranp. doesn't ass w/ COLLECTRIN
2).sml intestine: ACE2

Front 46

clinical manifestations of HARTNUP disorder: how linked to aa transp??

Back 46

dec Trp:
Rash with light sensitivity, psychosis, and cerebellar ataxia.

**Trp--> Niacin ( like pallegra)
Trp-->serotonin (neuro prob)

Front 47

4 Properties of senescent erythrocytes:
2 ways mac knows to eat them

Back 47

properties:

1*desialiated glycophorin
2**HEINZ bodies (denatured Hb)
3**SIDEROCYTES (Fe aggregates)
4*prog smler, less likely to clear splenic cap bed --> spelenomegaly

mac eating

1). Alt act mac:
-phosphatidyl serine on outer leaf
2).class act mac:
-damaged Hb binds N-ter of band 3 -->
hemichrome-band 3 & spectrin complex agg
--> IgG --> mac

Front 48

Haptoglobin vs Hemopexin:
-structural
-which is elevated in the
acute phase response??
**where are they degraded??

Back 48

Haptoglobin:
-binds Hb dimers (-->kid-->Hp deg to aa)
-a2-glygoprotein
-2L2H linked via 1 disulfide bond
**ACUTE PHASE PROTEIN

Hemopexin:
-binds heme&hemin (Fe3+) w/ high affinity
-B1-glycoprotein (-->liver -->hx-->bilirubin)

Front 49

Heme oxygenase
-location
-cofactor(s)
-by-product of rxn

Back 49

heme oxygenase:
-membrane-bound in er
-complex w/ NADPH cty P40 reductase
-prod 85% CO (measure of heme turnover)

Front 50

Heme oxygenase
3 isozymes
-which are inducible
-what induces them?

Back 50

CO1-inducible (prim in liver/spleen)
**hemin, insulin, epi, endotoxin, heavy metals, UV light

CO2- nervous system, intestine, endothelial cells
CO3- nervous system

Front 51

Heme oxygenase
by-product of rxn and what it induces

Back 51

CO~ HO produces 85% of it in the body
CO is a NT
CO stimulates guanylate cyclase

Front 52

Biliary obstruction:
what lab results indicate this
what will you find in the serum??

Back 52

delta-bilirubin ((bilirubin-gluconuride-albumin))
-normally NOT present AT ALL in serum
(inc direct/conjugated in serum)

-pale poop
-may have inc ID/UC bilirubin in serum (>1mg/dL)

Front 53

Neonatal Jaundice:
at what lab level of
UC/ID-bilirubin-albumin
do you get neural damage??

Back 53

20-25mg/dL
(1mg/dL is normal)

Front 54

PPAR-gamma-
**location**
complexed to what? what stim that?
-what stimulates it?? (natural + drug)
-effect of stim

Back 54

PPAR-gamma:
**adipocytes***
TZD/prostaglandins(PGJ2)/FA

RXRa: retinoic acid (vit A)

preadipocyte differentiation
inc adiopogenesis ('good' fat storage)
inc LPL
inc glucose uptake & utilization
dec hyperglycemia

Front 55

PPARa
location
what stimulates it

Back 55

PPARa
-liver/muscle
-fibrates stimualte it
-lipid ox (decTG, inc HDL-C)

Front 56

Things that adipocytes secrete:
3-cancer related
3-energy homeostasis
(1-4)-Acute-Phase Reactant
2-regualte vasculature

Back 56

cancer: TNFa, IL-C, Col VI
LPL
E homeostasis: leptin, adiponectin, resistin
Acute-Phase Reactant:
a1 glycoprotein, SAA3 PTX-3, 24P3
regualte vasculature- VEGF, monobutyrin

Front 57

Leptin

Back 57

leptin --> hypothalamic centers
--> effector systems (POMC & NPY neurons
---> food intake & E expenditure

Front 58

Adiponectin
-men or women have more adiponectin
2 receptors + location of each

Back 58

**anti-inflammatory
*reverses IR in mice
*protective
---females have more adiponectin---

-AdipoR1 --> muscle
-AdipoR2 ---> liver

Front 59

effect of TZD & IR, CVD, DIAII

Back 59

TZD --> PPAR-gamma
--> dec IR, dec CVD, dec DIA II

Front 60

TNFa & obesity
-effect on adipocytes

Back 60

TNFa
**inflammatory ~ ass w/ 'bad' M1 mac**
inc IR
inc TG lipolysis
inc FFA from WAT

Front 61

how do adipocytes respond to hypoxia??
(what TF?)
1). normal
2). high fat diet/obesity when cells can't keep up

Back 61

hypoxia -->HEFA & VEGF
1). angiogenesis, ECM remodeled

2).adipocytes can't keep up:
dead mac ---> bad M1 mac --> TNFa -->
(((more IR, more TG lipolysis, more FAA release)))
inflammatory cascade

Front 62

good vs bad mac
-names
-what they secrete
how are they activated>>??

Back 62

M1=bad mac
***stimulated by MPC1
((classically activated)
secrete TNFa ---> inflamm

M2=good mac
-alt activated
resp to phosphatidylserine

Front 63

OBESITY
-what cellular organelle is stressed?
this causes what?

LIVER - 1 term

Back 63

obesity:
-inc ER stress --> further dysregulation

liver: steatosis
-kupfler cell activation (mac in liver)

Front 64

Brite adipocyte

Back 64

BAT (UPC1 +) that arise w/in WAT
-tx obesity in the future??

Front 65

Phechromocytoma pt
((brown fat))

Back 65

adrenal tumor
=inc B-adrenergic sig
= inc BAT stim

Front 66

BAT
-location
-percent in pop: higher in men or women??
-people w/ BAT tend to have ___
****precursor shared w/ what*****

Back 66

BAT
-neck/scapular reg of adults
-higher in females (7.5%) -- PET scans
-people w/ BAT tend to have lower BMI
-BAT & myocytes share the same precursor (NOT WAT)