justin du bois department of chemistry stanford university · t u r n i n g t o x i n s i n t o t o...

10
T Turning Toxins into Tools for the Study of Ion Channels Justin Du Bois Department of Chemistry Stanford University Ischia Advanced School of Organic Chemistry Ischia, Italy 24 September 2014 Voltage-Gated Ion Channels: Remarkable Molecular Machines • transmembrane proteins that function to transport charged ions across lipid bilayer • found in all electrically excitable cells; respond to changes in membrane voltage • selective for K + , Na + , Ca 2+ , Cl ; ions pass at rates on the order of 10 7 per second! • crystallographic analysis of bacterial and mammalian K + ion channels The Voltage-Gated Sodium Channel 19Å cryo-EM image Sato Nature 2001 ~260 kDa α-subunit; functional expression in heterologous vectors α-subunit comprised of four repeat domains, each with 6 TM helices 10 gene loci encoding α-subunit identified in mammalian cells taken from King Channels 2008, 2, 100 Tools of the Channelologist electrophysiology immuno -cytology & histology fluorescent protein labeling fast and dynamic nature of neuronal processes necessitates methods that afford rapid temporal response

Upload: others

Post on 29-Jul-2020

6 views

Category:

Documents


0 download

TRANSCRIPT

Page 1: Justin Du Bois Department of Chemistry Stanford University · T u r n i n g T o x i n s i n t o T o o l s f o r t h e S t u d y o f I o n Justin Du Bois C h a n n e l s Ischia Advanced

TTu

rn

ing

To

xin

s i

nt

o T

oo

ls

fo

r

th

e S

tu

dy

of

Io

n C

ha

nn

el

s

Just

in D

u B

ois

D

epar

tmen

t o

f C

hem

istr

y S

tan

for

d U

niv

ersi

ty�

Isch

ia A

dva

nce

d S

cho

ol

of

Or

gan

ic C

hem

istr

y Is

chia

, It

aly

24

Sep

tem

ber

20

14�

Voltage-Gated Ion Channels: Remarkable Molecular Machines �

• transmembrane proteins that function to transport charged ions across lipid bilayer

• found in all electrically excitable cells; respond to changes in membrane voltage

• selective for K+, Na+, Ca2+, Cl– ; ions pass at rates on the order of 107 per second! • crystallographic analysis of bacterial and mammalian K+ ion channels�

The Voltage-Gated Sodium Channel

19Å cryo-EM image Sato Nature 2001�

• ~260 kDa α-subunit; functional expression in heterologous vectors

• α-subunit comprised of four repeat domains, each with 6 TM helices

• 10 gene loci encoding α-subunit identified in mammalian cells

taken from King Channels 2008, 2, 100�

Tools of the Channelologist

electrophysiology immuno -cytology & histology fluorescent protein labeling

fast and dynamic nature of neuronal processes necessitates methods that afford rapid temporal response

Page 2: Justin Du Bois Department of Chemistry Stanford University · T u r n i n g T o x i n s i n t o T o o l s f o r t h e S t u d y o f I o n Justin Du Bois C h a n n e l s Ischia Advanced

Sodium Ion Channel Modulators

H

Me

O

OH

MeN

O O

Me O

NHMe

Me

O

H

O

OH

OH

OHNHN

H2N

HO

HO

OHO

H

N NH

HN NH

NH2HO

HO

H2N

O NH2

O

O

MeO

O

H

MeMe

HMe

Me

AcOOH

OMe

OH

OHOMeO

Me

O

MeN

MeO O

Ph

O

O

O

O

O

O

O

OO

O O

Me

O

Me

Me

Me Me

Me

Me

H

H

H

H

H

HH H

H

H

H

HO

HH

H

H

CHO

tetrodotoxin

saxitoxin

zetekitoxin AB

pyrethrin I

batrachotoxin

aconitine

brevetoxin A

N

HN N

H2N

OHN

O

OH

NO

HO

HO

O

N H–O3SO

NH2

HO

A Total Synthesis of (+)-Saxitoxin – The Cliffs Notes Version�

N

NHBoc

OSitBuPh2

PMB O

H

3 steps from N-Boc L-serine ester�

Mbs = OMeS

O

O

NH

NH

NMbs

O NH2

ONH

MbsN

H2N

NH

NH

NMbs

O NH2

ONH

MbsN

H2N

N NH

NHHN

NH2

H2N

HO

HO

O

O

NH2

(+)-saxitoxin

N

NHBoc

OSitBuPh2

PMB OH

NH

MbsN SMe

1 step� 7 steps�

N NH

NHHN

NH2

H2N

O

O

NH2HO

β-saxitoxinol�

B(O2CCF3)3��

84%�

[O]��

>70%�

• desired target assembled in 14 linear steps from commercial material

• de novo construction provides access to a unique chemical probe�

HN

NHN

NMbs

HO HOO

NH2

MbsN

NH2

O

10 mol% OsCl3�oxone, Na2CO3�

57%�

Mbs = OMeS

O

O

Toxin Conjugates as Molecular Probes of NaV Function

H2N

N NH

HN NH

NH2HO

HO

O

O

NH

HN

OH2N

N NH

HN NH

NH2HO

HO

O

O

NH

NH3+

O

O

N

O

O

H2N

N NH

HN NH

NH2HO

HO

O

O

NH

+HN

O

N N

MeMeMeMe

Me

STX-Cy5

Fluorescent probes

pH = 9.5

H2O/CH3CN

STX-18F

PET imaging agents

STX-maleimide

Affinity labels STX-maleimide

Affinity labels

A ‘Library’ of Bis-Guanidinium Toxins

saxitoxin (STX) neosaxitoxin gonyautoxin 2

dc-gonyautoxin 1 hydroxybenzoate STX gonyautoxin 5

Lyngbya wollei toxin 3

STX ethanoic acid

Page 3: Justin Du Bois Department of Chemistry Stanford University · T u r n i n g T o x i n s i n t o T o o l s f o r t h e S t u d y o f I o n Justin Du Bois C h a n n e l s Ischia Advanced

An Asymmetric Synthesis of (+)-Gonyautoxin – Abridged

61% isolated yield single diastereomer

770%�N NH

NH

O

OTBDPSBF3•OEt2

CH2Cl2

N NH

NHHN

NH2

H2N

HO

HO–O3SO

O

O

NH2

gonyautoxin 2/3

7 steps�

N NH

N

O

OSitBuPh2

from L-serine ester in 4 steps

N NH

NH

NSO2OCH2CCl3

H2N

NC(O)CCl3

OTBDPS

4 steps�

N NH

NHHN

NC(O)CCl3

TcesN

H

OTBDPS

BF3•OEt2

Et3SiH

75%�

2 mol%Rh2(esp)2PhI(OAc)2

N NH

NHHN

NH2

H2N

O

O

NH2HO

HO

Second Generation Improvements to Streamline Synthesis

5 steps

N NH

NHHN

NC(O)OCH2CCl3

Cl3CCH2OSO2N

HO

HOO

O

NH2

N NH

NH

TcesN

H2N

N

O

CCl3

OSitBuPh2

N N

NH

TcesN

H2N

NH2

OSitBuPh2

N NH

NH

TcesN

H2N

N

O

OCH2CCl3

OSitBuPh2

N N

NH

TcesN

H2N

NH O

OCH2CCl3

OSitBuPh2

+ TrocCl

iPrNEt, CH2Cl2

[O]N NH

NHHN

NH2

H2N

O

O

NH2HO

HOHO

HO

11,11-dihydroxy-STX

recently isolated PSP from Alexandrium tamerense – Quilliam J. Nat. Prod. 2008, 71, 1518

H2cat. Pd/C

CF3CO2HMeOH

76%

10 : 1�1 : 6�

DMSO

DCCC5H5NH+ –TFA

88%

CH2Cl2

–OTf

NMeTrocN

Ph

885%�65%�

‘Unnatural’ Toxins: C10-Substitution

55%�

74%�

C10-modified GTX 3

A Highly Evolved Cork Stoppers Ion Flux

extracellular Domain I Domain II Domain III Domain IV

outer vestibule: E403 E758 M1240 D1532 selectivity filter: D400 E755 K1237 A1529

IV III I

N NH

HN NH

NH2HO

HO

H2N

O

O

NH2

D400K1237

D1532

E403

E758

E755

M1240

NaV1.1 NaV1.2 NaV1.3 NaV1.4 NaV1.5 NaV1.6 NaV1.7 NaV1.8 NaV1.9 NaX

SCN1A SCN2A SCN3A SCN4A SCN5A SCN8A SCN9A SCN10A SCN11A SCN7A

CNS CNS, PNS Brain, DRG, PNS Skeletal muscle Cardiac CNS, DRG, PNS DRG, SN, spinal cord DRG DRG Glial cells, DRG

���������������?�

TTX/STX sensitivity localization gene locus name

Novakovic Trends Neurosci 2001, 24, 473 Llewellyn Nat. Prod. Rep. 2006, 23, 200

Page 4: Justin Du Bois Department of Chemistry Stanford University · T u r n i n g T o x i n s i n t o T o o l s f o r t h e S t u d y o f I o n Justin Du Bois C h a n n e l s Ischia Advanced

A Comparison of Toxin Volume and Surface Potentials�

N NH

HN NH

NH2HO

HO

H2N

O NH2

O

(+)-saxitoxin

N NH

HN NH

NH2HO

HO

H2N

–O3SO O NH2

O

(+)-gonyautoxin III

O

OH

OH

OHNHN

H2N

HO

HO

OHO

H

(–)-tetrodotoxin

305 Å3 242 Å3 252 Å3

electrostatic surface potential –4 kT to +4 kT

Double Mutant Cycle Analysis to Study Protein Structure

WTNaV � STX

ΔΔGint = ΔG1 – ΔG2 – (ΔG3 – ΔG4)

ΔG1

WTNaV � MutSTX

ΔG2

MutNaV1.4 � STX

ΔG3

MutNaV � MutSTX

ΔG4

�  Magnitude of ΔΔGint varies with distance between two groups

�  Quantitative assessment of localized interactions

�  Previous studies limited by structural diversity of natural toxins used

-1.2

-1.0

-0.8

-0.6

-0.4

-0.2

0.0

70686664626058

ob

serv

ed

cur

rent

(nA

)

time (ms)

–100�

0�

–50�a

pp

lied

po

tentia

l (mV

)

Evaluating STX Block: Whole-cell Voltage-Clamp Recordings�

1.0 nM STX

5.0 nM STX

STX washed off

no STX

N NH

HN NH

NH2HO

HO

H2N

O NH2

O

(+)-saxitoxin

IC50 = 2.9 ± 0.1 nM against NaV1.4 (CHO)

Na+�

pipette

electrode

cell membrane

ion channel

N NH

HN NH

NH2HO

HO

H2N

O NH2

O

(+)-saxitoxin

IC50 = 2.9 ± 0.1 nM against NaV1.4 (CHO)

Na+��

pipette

electrode

cell membrane

ion channel

β-saxitoxinol

IC50 = 32 μM

N NH

HN NH

NH2HO

H

H2N

O

NH2

O

αα-saxitoxinol

IC50 = 559 nM gonyautoxin III

IC50 = 15 nM

N NH

HN NH

NH2HO

HO

H2N

–O3SO

O

NH2

ON NH

HN NH

NH2H

HO

H2N

O

NH2

O

11,11-dihydroxysaxitoxin

IC50 = 2.25 μM

structure-activity data for C11- and C12-derivatives challenge binding model �

Evaluating STX Block: Whole-cell Voltage-Clamp Recordings�

Page 5: Justin Du Bois Department of Chemistry Stanford University · T u r n i n g T o x i n s i n t o T o o l s f o r t h e S t u d y o f I o n Justin Du Bois C h a n n e l s Ischia Advanced

N NH

HN NH

NH2HO

HO

H2N

O

NH2

O

IIIIIV

D400K1237

M1240

D1532

E403

D758

E755

N NH

HN NH

NH2HO

HO

H2N

O

NH2

O

IIIIIV

D400K1237

M1240

D1532

E403

E758

E755

E758D

A Single Point Mutation Gives a Dramatic Result

IC50 = 2.9 nM for wild type NaV1.4

IC50 = 3.2 μM for E758D mutant

STX resistance in Mya arenaria ascribed to E→D mutation

Catterall & Trainer, Nature 2005

A Highly Evolved Cork Stoppers Ion Flux

extracellular Domain I Domain II Domain III Domain IV

outer vestibule: E403 E758 M1240 D1532 selectivity filter: D400 E755 K1237 A1529

IV III I

N NH

HN NH

NH2HO

HO

H2N

O

O

NH2

D400K1237

D1532

E403

E758

E755

M1240

NaV1.1 NaV1.2 NaV1.3 NaV1.4 NaV1.5 NaV1.6 NaV1.7 NaV1.8 NaV1.9 NaX

SCN1A SCN2A SCN3A SCN4A SCN5A SCN8A SCN9A SCN10A SCN11A SCN7A

CNS CNS, PNS Brain, DRG, PNS Skeletal muscle Cardiac CNS, DRG, PNS DRG, SN, spinal cord DRG DRG Glial cells, DRG

���������������?�

TTX/STX sensitivity localization gene locus name

Novakovic Trends Neurosci 2001, 24, 473 Llewellyn Nat. Prod. Rep. 2006, 23, 200

Human Pain Conditions linked to Sodium Channel Gene Mutations

Two autosomal dominant mutations in the NaV1.7 gene which cause a hyperpolarizing shift in activation resulting in redness, hyperalgesia, and burning pain�

Erythermalgia

Congenital Pain Insensitivity

NaV1.7 gene functional deletion - inability�to sense pain. �

Heckert, J. “The Hazards of Growing Up Painlessly,”

The New York Times Magazine Nov. 15, 2012

Page 6: Justin Du Bois Department of Chemistry Stanford University · T u r n i n g T o x i n s i n t o T o o l s f o r t h e S t u d y o f I o n Justin Du Bois C h a n n e l s Ischia Advanced

Human NaV1.7 is TTX ‘Sensitive’ and STX ‘Resistant’�

Time (s)

I/I 0

NaV1.4 control

+ 100 nM STX

Time (s)

NaV1.7 control

+ 100 nM STX

Time (s)

I/I 0

NaV1.4 control

+ 100 nM TTX

Time (s)

NaV1.7 control

+ 100 nM TTX

L L L L L L

M M M M M M

T T T T T T

Q Q Q Q Q Q

D D D D D D

F F Y Y Y Y

W W W W W W

E E E E E E

N N N N N N

T T T T T T

V V V I V V

L L L L L L

C C C C C C

G G G G G G

E E E E E E

W W W W W W

I I I I I I

E E E E E E

1.1 1.2 1.3 1.4 1.6 1.7

NaV Domain I Domain II Domain III Domain IV

A A A A A A

I I I I I I

T T T T T T

F F F F F F

K K K K K K

G G G G G G

W W W W W W

M M M M M T

D D D D D I

L L L L L L

T T T T T T

T T T T T T

S S S S S S

A A A A A A

G G G G G G

W W W W W W

D D D D D D

G G G G G G

N NH

HN NH

NH2HO

HO

H2N

O NH2

O

(+)-saxitoxin

N NH

HN NH

NH2HO

HO

H2N

–O3SO O NH2

O

(+)-gonyautoxin III

O

OH

OH

OHNHN

H2N

HO

HO

OHO

H

(–)-tetrodotoxin

toxin NaV1.4 Na

V1.4

M1240T Na

V1.4

D1241I Na

V1.4

M1240T

D1241I

NaV1.7 Na

V1.7

T1398M

I1399D

TTX 17.1 nM 466 8.7 90 18.6 5.0

STX 2.8 73 53 1153 702 2.3

GTX-III 14.9 228 38 1084 1513 22

A Possible Explanation For Differences in Toxin Affinity?�

Domain III Amino Acids Alter Pore Dimensions & Electrostatic Surface�

NaV1.4 NaV1.7

Site II – The Inner Pore

out

in

1

2

STX, GTX, TTX

Page 7: Justin Du Bois Department of Chemistry Stanford University · T u r n i n g T o x i n s i n t o T o o l s f o r t h e S t u d y o f I o n Justin Du Bois C h a n n e l s Ischia Advanced

V = 582 Å3 V = 502 Å3 V ~ 633 Å3

A Common NaV Receptor Site for all three Lipid-Soluble Neurotoxins?�

batrachotoxin�

H

Me

O

OH

MeN

O O

Me O

NHMe

Me

O

HAcO

OHOMe

OH

OHOMeO

Me

O

Me N

MeO O

OHO

OH

OHN

HO

Me

OH

HO

O

OMe

OMe

O

aconitine� veratridine�

Musutani, et al J. Pharm. Exp. Therap. 1981, 217, 812

Kosower FEBS Lett. 1983, 163, 161

Codding J. Am. Chem. Soc. 1983, 105, 3172

VVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVV ==================================== 5555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555555550000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000000222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222222 ÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅ333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333 VVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVV ~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~ 666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666666663333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333 ÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅ3333333333333333333333333333333333333333333333333333333333333333333333333333333333333

rmrmrmrmrmrmrmrmrmmrmmrmrmmrmrmrmrmrmrrmmrmmrrmmmrmmmrmmmrmrrmmmmmmmmrmmmrmm....... . . . . ... ExExExExExExEEExExExExExExExExExExExxExExExExExEExExExxExxxExxxxxEExxp.pppp.p.p.p.p.pp.pp.p.p.pppppppppppppppppppppppp.pppp.pp.ppp..p TTTTTTTTTTTTTT T TT TT TTT TTTTTTTTT T hhehehehehehehehehehehehehehehehehehhhehehhehehehehehehhhhehehehehehehehehheheeeheeheeheeeeeeeeheeeeeeheerararararrararararaararararaaaaaaaraararaaaararaaararrrraraaaararaaaaraaraaarraaaaaaraaaaaaraaraaaaaaaaaaaaraaaaaaaapp.p.pppp.pppppp.pppppppp.pppppp.pppppppppppppppppppppppppppppppppppppp.pppp.pppppp.pppp.pppp.p.ppp..pp.p 1919191919191919191919999999999999191999999991919191999991919199991919991991199991919999119991199199999919999999999999999991999999999999999999999999999998818888888188881818818181818181818188818181888881881881818188181888818881818188818188888188188181888188818181881811818881111881818111818111118118818818181888888888188888888 ,,,,, ,,,, ,,,, ,, ,,,, ,, ,,,,, 2212122222221212121222121212222222212212121222212122121222222221212121222222221222222222222222222222122122222222222222222222 7777777777777777777777777777777777777,,, , , , , , ,,,,,,, 8181818181818181818818181818181818181881118111811111112222222222222 2 2 22 2222 222 2222222222222 222222 2222

ooooooooooooooooooooooooooooooooooooooooooooooooooooooooooooooooooooooooooooooooooooososososossssossososoooososoosososososssososoooooooosoososossooooooooososoosososososooooooosssoooooosoosoosoooossoooooooooosooooosooooooooooosssoooooooooosssoooooooossoooossssooosooosoooosososoosossososssosooossossssoosoosossoosoooosossoossosoosowwewewwwewewewewewewewwewewewwweweeewewewewewweweweweeeweweweeweeweewewwwewewewweeeewweeeeewewwwewwewewwewwwwwwwwwww rr r rrrrrrrrrrrrrrr rrrrr rrrrrrrrrrrrrrrrrrrrrrrrrrrrrrrr rrrrrrrrrrr FEFEFEFEFEFEFEFEFEFEFEEEFEFEFEFEFEFEFFEFEFEEFEEEEEFEFFEEEFEEEEEFEFEEEFFEFEEEFFFFFFEEEFEEFFFEEEFFFFFEFFFEFFFFEEEEEEEEEF BSBSBSBSBSBSBSBSSBSBSBSBSBSBSBSBSBSSBSBSBSBSBSBSBSSBSSSBSBSBSSSSSSSSSBSSSBSSSBBBSSSSBSBBSSBSBSSSBBSBBSSSSSSSBSSSSSS LLLL LLL LLLLLLLL LLLLLLLLLLLLLLLL LLLLLLLL LLLLLL L LLLLLLLLLLLLLLLLLLLLLLLLLLLLLeeeteeteeteteteteteeettttetttetetetetttttteeeeteetttetttetetettetteteetetteteeettteteeeetettetteeteteteeteteeteteteeeeteeeeeteeeeteeeteeeeeeteeeteeetteeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeettteeeteeeeeeeeeeeeeeettteeeee ttttttttttttttttttttttttttt.t.tt.tttt.t.t..t.t.t.t.t.ttttt.t.tttt.. 1919191919191999999999991911911919191911999919199191999191919191199991991919999191919911119999199199191999999199919191999199199191999919191919911999911919199111999111991991919199191991919919911919838383838383838383838383833883338838883888888883888888888888883883838888838383888883888838883838383888388888383883888838333833338383838888383838838338833838833338383,, , ,,,, ,, , ,, 1616161616161616166616661116611116111616116661616161666666661666666666666666666666666616666666666666666666666666666666666666666333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333,,,,,, ,,, , , , , , ,, , ,, , 1616616661666666661161616161666161611161116666616161611616166611616166661616666166661666661661666166666666666666666611161616661611611666611116666661111166616666111161166616666666611666666111166666111666166161616666666611111111111111111111111111111111111111111111111111111111111111 111111111111

mmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmm CCCCCCCCCCCCCCCCCCCCCCCCCCCCCCCCCCCCCCChehehehehhhehehehhheheheheheehehehehhehehheheeeheheheheeeeheheeheheeehheeheeehehhhheeeehehehhheheeeehehhhheehehehehhheeeeeehhhheheeeeheeheeheeeeeeeeeheeeeeeheeehehhheeeehhhhehehehhhhehhhhheeheeheh mmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmmm SSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSooococococococoococococococococococccooocococococcocococooococcococococococoococococcoocoocccoooooooocccooooococoococcocccccccocccc 191919191919191999999191991999199199199919191919999919999991919199191919199911999191991911919191919911911999999191999911999199199998838383838383838383838383833383883888338388883383888888883883838383888883838388333383883833383333338338338383383383838388383 101010101010100000010010101010001010101001000001001111001000011010111000000010100055555555555555555555555555555555555555555555555,,, 31313131313131313131131331313133331111313333333 727272727727272222222222722222222722222227272722222222222722222222222777222222222222222222222222222727222222227272222222222272727222222272222777272222222272222727222222222727277222222222272272222222227222222222222272272722227272227277772

VVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVVV ================================================================================================================================================================ 55555555555555555555555555555555555555555555555555555555888888888888888888888888888888888888888888888222222222222222222222222222222222222222222222222222222222222222222 ÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅÅ33333333333333333333333333333333333333333333333333333333333333

MMMuMuMMMMuMuMuMuMMMuMMMMMMMMuMuuMuMuuMuMuMuMuMuuMuMMuMuMuMuMuMuMuMMuMMMuMMMMMuMuuMuMuMuMMuMuMuMuMuMMuMMuMuMuMMMMuuuMuMMMMuMuMMMuMMMuMuMMuMuMuMuMuMuMMMuMuMMMMuMMMuMuMMMuMMMuMuMuuMMMMMMMuMMMuMMMuuMuMuMuMuMuMMMuMMMuMMMMuMuMuMMMMuMuMuMuMuMuMMMMuMMuMuMMuMuMuMuMMMMuMuMuuMuMuMuuuMMuMMuMMuMMMuMuMuMuMMMMMMuMuMuMuuMuMMMMMuMMuMuMMMMMuMuMuMuMMMMMMMMuMMuMuMMuMMMMuMMMMuuuMMuMuMMMuMuMuMMMuMMuMMMMuMMMMMMMuMuMMuMuMMMMMuMMMMMMMMuuuMMMMMMMMMMMMuMMMMMMMMuMuMMMMMMuuMMMMuMMuMMuMMMMMMuuMMMMuuMMuMMMMuMuMuMuMMuMMMMMuMMMMMMMMMuuMuMMuMMMMMuMuuuuuMuMMMMMMuMuMuMuMMMuMMMMMuMMuMuMMMuMuuuMuMuuMuMuuMuuMMMMuMuMuuMMuuuuuuMuMuMuMMMMMMMuMuMuMuMuuuuuuuMuuMuMMMMMMMMMMMMuMuMMuuMuMuuuuuMMMMMMMMMMMMMuMMMuMuMuuuuMMMMMuMMMuuuuuuuuuMMMMMuMMMMMMMMMuuuuuuMMuMMMMMMMuuuuuuMMMMMuMuMMMMMMMuuuuuuuuuuusususussussssuuususussssssssuuuuuuuusssssuuuuuuuuuuuuuuusussusssuuuuuuuuuuusssuuuuuuuuuuuuusussuuuuuuuuuuuuususususussusuuusuuusuuuususssususssusuuuusuusuuusuuuuuuuususususssusuuuuuuuuuuuuusssussususuuususuuusuuuusususussusssssuuuusuusssssssssusuuususuuusussssssssssusususuuuuuuuuuuuuuuususuusssuuuuuuuuuuuuusuuusssssssuuuuuusuuusssssssssuuuuususussssuuuuuuusususssssuuuuuuuuuuussssssusuuuuuuuuussssssssuusuuuuuuususssssuusuuuuuuuusssssussuusuuuuuuuuusssssusssssssusuuuuuuuussssssssuuuuuuuuuuuuussssssssuuuuuuuuuuussssssssssuuuusssssssuuusssssssuussssutttattattttttttttattataaataataataatatatatattttttttttatttttatatattataaaataaatatatatttttttttttttttatatatatatataatatatttttttttttattaatatataaatataaatttttttttttttttaatatataatataatataatattttttattattatataaataaatataaattttttttattataaatataataaaataaaatatatatttatatatatatatatataaaatatatatataattttataatatatatatatataatatatataattttttaaaaaatattttttaatataataatatttttttaataattttttataatatatttttttattaaataaatatatttatattatattaataattttattttattataatttttatataaaaaaatatttttatatattaaaaaaaataaataaatttatatattaatataaaaaaaaaaattttttataaaaaaaaaatataattttttaaaaaaaaaaaaaaattttttttttaaaataaaaaaaattttaaaaaaaaaattttatattatttaaaattattatttaaaaaatatttaaaataaaaatttataaaaaaaaaataaaatttaaaaaaaaaaaaaaaaatttttaaaaaataaaaaaatttaaaaaaaaaatattaaaaaaaaaattaaaaaaaaaaaataaaaaaaaaaaaaaaaaaaaaaaaaaaaataaaaaaaattaaaaaaaaaaaaaninninninininininnnnninnnnininiininiinninininininnnnininininnnnnininnniiniinnininnniniiniinnnniinninininnnnnnninininnnnninninnnnninnnnnninnnnnnnnnnnininninnnnnnnnnnnniinnnnninnnnnnnnnnnnininininnniinnnininnnnnnnnnnnnnnnninnnnnnnnnnnnnnnnnnnnnnnnnnnnnnnnnininnnnnnninnnnninnnininnnnnnnininnnnnninninnnnnnniiiiinnnnnnnininnnniiinnnnnnnnnnnnnniiiiiiinnnnnnnniiiiiininnnnnnniiiiiininnnnnnnnniiiiinnnnnnnnnnnniiiiinnnnnnnnnnnnnniiinnnnnnnnnnnnnnnii, ,,,,,,,,,,,, , ,,,,,,,,,,,,,,,,, ,,,,,, ,,,,,, ,,, ,,,,,,,,,,, eteteeteeeeetetetetettttteteteteteteetetttteteteteteteeeeetteteteteteteteettttettetetetetetetetetetetteteteteteteteetetetetettetetetetetetetettteteteteteteteteteetteteteteteeeteeeeetetteteteteeteeeteteetetetetettteeeeeettteteteteteetetteeteteetteteteteteteeteetettttettteeeeeeetetetetettttetteettttteteteteeeetttttteeteeeetttetettteteeeeeeetttteeteeettttttttteteeetettetetetteteteeeeteettttttteteteetettttteteeteetteeeettteeeeeeeeeettetteeeeeeetetet aaaaa aaaaaaaaaaaaaaaa aaaaaaaaaaaaaaaaaaaaaaaaaaaaa aaaaaaaaaaaaaaaaaaaa aaaaaaaaaaaaaaaaa aaaaaaaaaaaaaaaaa aaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaa aaaaaaaaaaaaaa a aaaaaa aaaaaaaaaaaaaaaaaaaaaaaaaaaa aaaaa a aaaaaaaaaaaaaaaaaaaaaaaaaa aaaaaaaaaaaaaaaa a aaaa aaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaa aaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaa aaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaaa aaaaaaaaaaaaaaaaaaaaaa aaaaaaaaaaaaaaaaaaaaaaaaaaa aaaaallllllllllllllllllllllllllllllllllllllllllllllllllll lllllllll JJJJJJJJJJJJJJJJJJJJJJJJJJ.JJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJ.JJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJJ.JJJJJJJJJJJJJJJ.JJJJJJJJJJJJJJJJJ.JJJJJJJJJJJJJJJJ.JJJJJJJJJJJJJJJJJJJJJJJJJ.JJJJJJJJJJJJJJJJJJ.J.JJJJJJJJJJJJJJJJJJJJ.J.JJJJJJJJJ.JJJJJJJJJJJJJJ.JJJJJJJJJJJJJJ.JJJJJ.JJJJJJJJJJ.JJJJJJJJJJJJJJ.JJJJ.JJJJJJJJJ.JJJJJJJJJJJJJJ.JJJJJJJJJJJ.J.JJJJJJJ.JJJ.JJ.JJJJJJJJ.JJJJJJJJJJJJJJJJJJJJJJ.JJJJJ.JJJ.JJJJ.JJJJ.JJ.JJJJJJJJJJJJJJ.J.J..JJJJJ.JJ.J.J..J.JJJJ...JJJJJJ.J.J.

CoCoCoCoCoCoCoCCoCCoCCoCoCCCCCCCoCoCoCoCoCoCoCoCCoCoCoCoCCCCCCCCCoCCoCCoCoCoCoCoCoCCoCCoCCoCoCCCCCCCCooCoCCoCCCCoCCCCCoCCCoCoCoCCoCCoCoCoCoCoCCCCCoCCoCoCoCCoCoCoCoCoooCoCoCoCCCoooCoCoCooooooCooCoCCCCoCooCCCCCCooCoooCCCCCooCoooCCCCCCCCCCCCCoCoCooCoCCCCCooCooCoCoCoCooooCCoCCCCCCCCCCCooCoCoooCoCoCCCCCCCCCoCCoCooooCCoCoCoCCCCCoCoooCooooCoCoCoCoCoCCCCoCoCoCCoCCCCoCoCoCCCoCooCooCoCCCoCooooCooCCCoooCCoCCCoooooooooooooCCCooooooddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddddd iniiininininnininininniniiininiiiinnininnniniiininnnnininnnnnninnnininnnnnnnininininnnnnninnnnninnnnnnninnnnnnnnnnnnnnnnggggggggggggggggggggggggggggggggggggggg

A Common NaV Receptor Site for all three Lipid-Soluble Neurotoxins?�

batrachotoxin�

H

Me

O

OH

MeN

O O

Me O

NHMe

Me

O

HAcO

OHOMe

OH

OHOMeO

Me

O

Me N

MeO O

OHO

OH

OHN

HO

Me

OH

HO

O

OMe

OMe

O

aconitine� veratridine�

O��O� O�

2.4 �

4.5 �

2.8 Å*�

Musutani, et al J. Pharm. Exp. Therap. 1981, 217, 812

Kosower FEBS Lett. 1983, 163, 161

Codding J. Am. Chem. Soc. 1983, 105, 3172

avg. distance to N ~6.0 �

holding open a lysine gate?�

NH

HH

+O

OO

H3N+

A Common NaV Receptor Site for all three Lipid-Soluble Neurotoxins?�

batrachotoxin�

H

Me

O

OH

MeN

O O

Me O

NHMe

Me

O

HAcO

OHOMe

OH

OHOMeO

Me

O

Me N

MeO O

OHO

OH

OHN

HO

Me

OH

HO

O

OMe

OMe

O

aconitine� veratridine�

electrophysiology recordings (current vs time) reflect differences in mode of action

The Venom of the Columbian Arrow Poison Frog�

batrachotoxin (BTX)�

Phyllobates terribilis

• first isolated from phyllobates aurotaenia (1965) (family Dendrobatidae) • 5000 frogs delivered 27 mg of pure toxin • structural elucidation/pharmacological evaluation by Daly and Witkop (NIH) • mouse LD50 (subcutaneous) = 2 μg/kg

Pitohui dichrous (Papua New Guinea) Daly & Witkop Science 1971, 172, 995

homobatrachotoxin�H

Me

O

OH

MeN

O O

Me O

NH

Me

O

H

Me

Page 8: Justin Du Bois Department of Chemistry Stanford University · T u r n i n g T o x i n s i n t o T o o l s f o r t h e S t u d y o f I o n Justin Du Bois C h a n n e l s Ischia Advanced

batrachotoxin�

NMe

O

O

NH

Me

Me

O

HO

Me

HOH

Me

O

Designing A Synthesis of Batrachotoxin – Prior Art �

Kishi J. Am. Chem. Soc. 1998, 120, 6627�Wehrli Helv. Chim. Acta 1973, 56, 139�

O

Me

Me

H H

H

OMe

H

HO

Me

H

O

OH

Wehrli (1973) Kishi (1998)

NMe

OH

O

HO

Me

HOH

Me

O

batrachotoxinin A�

NMe

OR

HO

HO

Me

OH

Me

O

HNMe

OR

HO

HO

Me

OH

Me

OHA

C D

A Truncated Form of Batrachotoxin

batrachotoxin

R =

final step esterification affords C/D/E ring analogue structures

H

Me

O

OH

MeN

O O

Me O

NHMe

Me

O

H

Electrophysiological Analysis of BTX C/D/E Analogues

IC50(μμM)

81.4 ± 4.8 41.4 ± 1.0 38.8 ± 1.0 17.4 ± 0.4

R =

19.2 ± 2.4 11.1 ± 0.3

–100

0

–50

0.0

–0.2

–0.4

–0.6

–0.8

–1.0

–1.2

58 60 62 64 66 68

ob

serv

ed

cur

rent

(nA

)

time (ms)

ap

plie

d p

ote

ntial (m

V)

70

control 200 μM CDE-naph

0.0

–0–0–0–0–00–0000000–0000000000–000–000–00––0000.222222222222222222

–0.4

–0.6

–0.8

–1.0

–1.2

58 60

ob

ob

ob

obb

obb

obb

ob

ob

ob

ob

obb

ob

obb

ob

ob

ob

obb

ob

obb

ob

ob

ob

ob

ob

ob

ooooooooooooseseseseseseseeseseseseseseseseseseseseseeseeseseseeseeseeeeseeeeessss

rvrvrvrvrvrvvrvvrvvrvvrvvrvvrvvrvvrvvrvvrvvvvrvvrrrrrre

dde

de

dde

de

de

de

de

de

ddde

de

dde

de

dde

de

dde

de

ddde

ddde

de

de

de

de

ddde

de

deeeeee

ccccccccccccccccccccccccccccccccccccururururrururururrurrururururrururururururrrrururururrurrururururururrrrururrurrururrrrrruuuuuuuuuuuuuuuuuu

rerererereerererererererereeerereeererereeeeererereererereeeereeereerererereeeeererereeereeereeerrrrrrrrntntntnttntntntntntntntntntntntntttntntntntttntntntntnttntnttntntnttntntntnttttnttnnnnnnnnnnnnnnnnnnnnnnn

(((((((((((((((((((((((((((((((((((((((((((((((nAnAnAnAnAAnAnAnAAnAnAnAAAAAAnAAAAnAAAAAnAnAnnnnnnnnnnnnnnnnnnnnnn

25 μM CDE-naph 5 μM BTX

Relative Potency of CDE-naph Against NaV Subtypes�

CDE-naph

NaV isoform IC50 (µM) Stdev

rNaV1.2 30.1 ± 6.9

rNaV1.4 17.4 ± 0.4

hNaV1.5 19.3 ± 3.5

hNaV1.7 7.8 ± 1.7

Minimal differences in IC50 between subtypes

S6 Residues that line inner pore > 96% conserved

CDE-naph (M)

Rela

tive

cur

rent

(%

)

CDE naph

Page 9: Justin Du Bois Department of Chemistry Stanford University · T u r n i n g T o x i n s i n t o T o o l s f o r t h e S t u d y o f I o n Justin Du Bois C h a n n e l s Ischia Advanced

IC50(μM) 13.8 ± 0.7 15.4 ± 0.6

Mirror image isomers display nearly identical potency

Through the ‘Looking Glass’�

Single Point Mutagenesis Experiments with NaV1.4

CDE-naph

300

200

100

0

IC50

(μμ

M)

D1 D2 D3 D4

D1 D2 D3 D4

Homology Model of the Inner Pore (NaVMs)�

Catterall et al. Nature 2011 Clapham, Yan Nature 2012 Wallace et al. Nature 2013

out

in

Minimal differences in IC50 between subtypes

S6 Residues that line inner pore > 96% conserved

N434

F1579

N1584

Page 10: Justin Du Bois Department of Chemistry Stanford University · T u r n i n g T o x i n s i n t o T o o l s f o r t h e S t u d y o f I o n Justin Du Bois C h a n n e l s Ischia Advanced

Rationalizing the Lack of Enantiospecificity��  binding is state-dependent to open channel

�  hyperpolarizes threshold activation by 30–50 mV

�  eliminates inactivation gating

�  reduces single channel conductance by ~60%

�  modifies Na+ ion selectivity

H

Me

O

OH

MeN

O O

Me O

NHMe

Me

O

H

Many Outstanding Questions Remain�

batrachotoxin

Acknowledgements

Collaborators

Dr. Sandip Biswal, Aileen Hoehne, Deepak Behera — Radiology

Prof. Merritt Maduke — Mol. & Cell. Physiology

Prof. W. E. Moerner, Hsiao-Lu Lee, Shigeki Iwanaga — Chemistry

Prof. Vijay Pande, Paul Novick — Chemistry

Prof. David Yeomans — Anesthesiology

Dr. J. Zablocki, Dr. M. McGregor — CVT/Gilead

Support

National Institutes of Health

Pfizer

Center for Molecular Analysis and Design

SiteOne Therapeutics, Inc.

Brian Andresen

Matt Axtman

Rosemary Conrad

Sloan Devlin

Darren Finkelstein

James Fleming

Andrew Hinman

Arun Thottumkara

James Walker

Dr. Matt McReynolds

Dr. Alan Whitehead

Dr. Alison Ondrus

Dr. Fred Menard

Dr. Tatsuya Toma

Matt Logan

Jeff Merit

John Mulcahy

William Parsons

Heather Roberts

Rhiannon Thomas

Zahra Taji

Bio-X Stanford University

Stanford Dean’s Fellowship

NSERC Canada

Japan Society for the Promotion of Science