George Scangos, CEO of Biogen Idec, earned $11.3m in 2011 – reflecting the US biotech company’s runaway success.

Partnerships with big pharma companies such as Genentech, UCB and Isis Pharmaceuticals, as well as many smaller biotech firms, have helped Biogen Idec reach an all-time share value peak of $127.57.

Since taking over as CEO in June 2010, Scangos has restructured the company and reduced its R&D costs while realising the commercial potential of biologics such as Tysabri (for MS) and Rituxan (for non-Hodgkin’s lymphoma).

Scangos’ $11.3m pay package for 2011 includes $1.2m in salary, a $2.7m bonus and $4.4m from the vesting of stock options.

Other members of Biogen’s executive board were also rewarded: Francesco Granata, former head of commercial operations, earned $624,000 in salary plus a $373,200 bonus; Steven Holtzman, head of corporate development, earned $600,000 and a bonus of $392,040; and Doug Williams, head of R&D, earned $630,000 and a $456,221 bonus.

The world’s oldest biotech company, based in Biogen Idec started as Biogen in 1978 and merged with Idec (which had formed in 1991) in 2003.

US biotechnology company Biogen Idec has agreed to pay up to $299m (including milestone payments) for the rights to a drug for spinal muscular atrophy (SMA), the foremost genetic cause of infant mortality.

ISIS-SMNRx is an investigational drug from Isis Pharmaceuticals that has been granted orphan drug status by the FDA for the treatment of SMA.

Biogen Idec and Isis Pharmaceuticals have formed an exclusive global option and collaboration agreement to develop and commercialise the drug.

Isis will receive an upfront payment of $29 million and up to $45 million in milestone payments before the drug is licensed, then up to a further $225 million in regulatory milestone payments, as well as royalties.

Isis will be responsible for the clinical development of ISIS-SMNRx through to the completion of phase III clinical trials. Biogen Idec will be responsible for the drug’s global development, regulatory approval and commercialisation.

SMA is a genetic disorder that causes muscle atrophy and weakness, affecting one child in every 10,000. In its most severe form, SMA reduces a child’s life expectancy to about two years.

ISIS-SMNRx is an ‘antisense’ genetic therapy that alters the splicing of a closely related gene to compensate for the defective gene that causes SMA.

“SMA can kill children before their second birthday and there are currently no therapies to treat the disease,” said George A. Scangos, CEO of Biogen Idec. “The unmet need could not be any greater, and Isis’ antisense compound has the potential to be a highly effective, first-to-market therapy for this deadly disease.”

Stanley T. Crooke, CEO of Isis, commented: “Biogen Idec’s expertise in the global development and commercialisation of innovative new therapies for neurologic diseases is a great strategic fit to advance ISIS-SMNRx.”

Genzyme and Isis Pharmaceuticals have submitted a marketing authorisation application to the EMA for the approval of Kynamro (mipomersen) to treat homozygous and severe heterozygous familial hypercholesterolemia.

Kynamro is a first-in-class apo-B synthesis inhibitor currently in late-stage development for the reduction of LDL cholesterol (LDL-C).

Paula Soteropoulos, Vice President and General Manager of Genzyme’s Cardiovascular Business, says the submission is a “significant step in the development of mipomersen”.

The US regulatory submission of inhibitor is now planned for later this year.

Familial hypercholesterolemia is a genetic disease that results in increased LDL-C levels and can lead to premature heart disease and heart disease-related death.

Patients with severe familiar hypercholesterolemia have cholesterol levels two-to-four times higher than recommended levels, even when taking multiple cholesterol-lowering medication.

“Mipomersen has the potential to change the management of patients with homozygous and severe heterozygous familial hypercholesterolemia,” said Stanley Crook, Chairman and CEO of Isis Pharmaceuticals.