Sept 24(Reuters) - The U.S. Food and Drug Administration

approved IntraBio's drug for a rare and fatal genetic disorder,

the health regulator said on Tuesday, just days after the agency

cleared the first treatment for the disease.

Niemann-Pick disease type C (NPC) is a rare genetic disorder

that affects the nervous system and other organs, causing

physical and mental disabilities such as speech issues,

difficulties with swallowing and coordination, clumsiness and

others over time.

NPC can occur between infancy and adulthood, which is why it

is sometimes called "childhood Alzheimer's disease." People

affected by it only live for about 13 years.

The company estimates that NPC affects one in 100,000 live

births.

On Sept. 20, the FDA approved Zevra's drug,

Miplyffa, for the treatment of NPC - the first treatment to get

a nod for the condition.

The FDA approved the drug for the treatment of neurological

symptoms associated with NPC in adults and pediatric patients

weighing at least 15 kilograms.

IntraBio's oral drug, called IB1001, will be sold under the

brand name Aqneursa. However, the regulator did not disclose the

price of the drug.

On Monday, Zevra said it would price its rival treatment,

Miplyffa, at a wholesale acquisition cost of between $40,000 and

$106,000 per month, depending on dosage.

Aqneursa should be taken orally up to three times per day,

with or without food. The recommended dose varies depending on

the individual's body weight.

The approval was based on a late-stage clinical trial in 60

patients that showed the drug helped reduce symptoms compared to

a placebo.

The FDA also put out a warning that Aqneursa may cause

embryo-fetal harm if used during pregnancy.

The Austin-headquartered company said in March it had raised

over $40 million in equity funding to support the

commercialization and launch of IB1001, subject to FDA approval.