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under this collaborationby nearly $35million. Under the terms of the amended agreement,we are eligible to receive up to $303.8
million in a license fee andpayments, including$78.8million inmilestone andother payments associatedwith the clinical
development of ISIS-SMN
Rx
prior to licensing and$150million inmilestone payments if Biogen Idec achieves pre-specified
regulatorymilestones.
As ofDecember 31, 2013, we had earned$7million inmilestone payments for advancing the ISIS-SMN
Rx
Phase 2program.
In addition, basedon the further advancement of ISIS-SMN
Rx
Phase 2program, Biogen Idecwill payus $9.3million in the first
quarter of 2014.Wewill earn the nextmilestone payment of $18million ifwe dose the first patient in thePhase 3 study in infants
withSMA, which is designed to supportmarketing registration for ISIS-SMN
Rx
in theUnitedStates andEurope.
ISIS-DMPK
Rx
In June 2012,we andBiogen Idec entered into a second and separate collaboration and license agreement todevelop and
commercialize a novel antisense drug targetingDMPK for the treatment ofDM1, ISIS-DMPK
Rx
. We are responsible for global
development of the drug through the completionof a Phase 2 clinical trial. Biogen Idec has the option to license the drug through the
completion of the Phase 2 trial. Under the terms of the agreement, we received an upfront payment of $12million, whichwe are
amortizing through June 2017. Over the termof the collaborationwe are eligible to receive up to$259million in a license fee and
substantivemilestone payments. InOctober 2013, we earned a $10millionmilestone paymentwhenwe initiated an IND-enabling
toxicology studyon ISIS-DMPK
Rx
, andwe are eligible to receive up to another $49million inmilestone payments associatedwith the
development of ISIS-DMPK
Rx
prior to licensing. We are also eligible to receive up to$130million inmilestone payments if Biogen
Idec achieves pre-specified regulatorymilestones. In addition, we are eligible to receive up to double-digit royalties on anyproduct
sales of the drug.Wewill earn the nextmilestone payment of $14million ifwe initiate aPhase 1 study for ISIS-DMPK
Rx
.
Neurology
InDecember 2012, we andBiogen Idec entered into a third and separate collaboration todevelop and commercialize novel
antisense drugs to three targets to treat neurological or neuromuscular diseases. We are responsible for the development of the drugs
through the completion of the initial Phase 2 clinical study. Biogen Idec has the option to license a drug from each of the three
programs through the completionof Phase 2 studies. Under the terms of the agreement, we received anupfront payment of $30
million, whichwe are amortizing throughDecember 2020. Over the termof the collaborationwe are eligible to receive up to $259
million in a license fee and substantivemilestone payments per program. We could receive up to$59million indevelopment
milestone payments to support research anddevelopment of eachprogram, including amounts related to the cost of clinical trials, and
up to$130million inmilestone payments ifBiogen Idec achieves pre-specified regulatorymilestones. In addition, we are eligible to
receive double-digit royalties on anyproduct sales of drugs resulting from eachof the three programs. Wewill earn the next
milestone payment of $10million ifwe initiate an IND-enabling toxicology study for a development candidate identifiedunder this
collaboration.
StrategicNeurology
InSeptember 2013, we andBiogen Idec entered into a fourth and separate collaboration, which is a long-term strategic
relationship focusedon applying antisense technology to advance the treatment of neurological diseases. As part of the collaboration,
Biogen Idec gained exclusive rights to the use of our antisense technology todevelop therapies for neurological diseases andhas the
option to license drugs resulting from this collaboration. The exclusivity for neurological diseaseswill last six years, andmay be
extended for any drug development programs being pursuedunder the collaboration. Under the terms of the agreement, we received
anupfront payment of $100million and are eligible to receivemilestone payments, license fees and royalty payments for all drugs
developed through this collaboration, with the specific amounts dependent upon themodality of themolecule advancedbyBiogen
Idec. Ifwe have a change of control during the first six years of the collaboration, wemaybe required to refundBiogen Idec a portion
of the $100millionupfront payment, with the amount of the potential refunddecreasing ratably aswe progress through the initial six
year termof the collaboration. We are amortizing the $100millionupfront payment throughSeptember 2019. Because the
amortizationperiod for the upfront payment will never be less than the initial six year termof the collaboration, the amount of revenue
we recognize from the upfront payment will never exceed the amount that Biogen Idec couldpotentially require us to refund.
If an antisensemolecule is chosen for drugdiscovery anddevelopment of a neurological disease, we are eligible to receive up
to approximately$260million in a license fee and substantivemilestone payments for each antisense drugdevelopedunder the
collaboration.We are eligible to receive up to approximately$60million for the achievement of research and developmentmilestones,
including amounts related to the cost of clinical trials, andup to$130million for the achievement of regulatorymilestones.Wewill
usuallybe responsible for drugdiscovery and earlydevelopment of antisense drugs andBiogen Idecwill have the option to license
antisense drugs after Phase 2proof of concept. Biogen Idecwill thenbe responsible for later phase development and
commercializationof the licenseddrug. In addition, we are eligible to receive double-digit royalties on anyproduct sales of antisense
