arterial calcification, generalised, of infancy type 1

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arterial calcification, generalised, of infancy type 1

An autosomal recessive disorder (OMIM:208000) characterised by calcification of medium to large arteries, myointimal proliferation resulting in arterial stenosis, and focal periarticular calcification. Most die in early infancy from myocardial infarction, congestive heart failure or hypertension due to vascular occlusion.

Molecular pathology
Caused by loss of function mutations in ENPP1, which encodes a transmembrane glycoprotein that regulates soft tissue calcium and bone and joint cartilage mineralisation by generating pyrophosphate (PPi), a physiological inhibitor of hydroxyapatite crystal formation and a suppressor of chondrogenesis.
References in periodicals archive ?
We have a long-standing relationship with IIAC and are very pleased to be partnering with them again to meet their expanding security needs.
Incheon has thrived by using the latest and most innovative technology in all stages of the passenger journey, from check-in to immigration, and Rockwell Collins ARINC suite of products has played a major role in that over the years, said Seong Kag Hong, vice president, Aerodome Division of IIAC.
Rockwell Collins will also integrate its ARINC vMUSE common-use platform and other passenger and baggage processing solutions with IIAC s new AirCUS check-in solution, offering airlines greater flexibility and reliability while ensuring smooth passenger flows and operational efficiencies.
IIAC s knowledge of airport operations and our expertise in developing cutting-edge, scalable passenger processing solutions create a perfect team for this endeavor, explained Heament John Kurian, managing director, Information Management Service, Asia Pacific for Rockwell Collins.
IIAC Acting President and CEO Hong-Yeol Choi presented CEB with the 2013 Best Airline Service Award in a ceremony held recently.
According to IIAC, they selected the leading airlines among airlines which have operated regular flights to Incheon Airport for more than two years, and have performance of more than 50,000 passengers per year.
Jung Tae Song, Operations Manager of IIAC said: "We are implementing the kiosks as part of the Automated Information System which plays a key role in making the airport a fully intelligent terminal for all airport users and Travellers".
Incheon InternationalAirport Corporation- Based in Seoul South Korea, IIAC opened for business in 2001.
Many respondents want this information summarized in a one-page document, BetterInvesting's IIAC said.
It added that consortium also including four Seoul construction firms will construct the airport by 2018 and the IIAC will be permitted to manage the property up until 2067.
The IIAC functions South Korea s main hub, which controlled approximately 40 million passengers previous year and has been quite a few times regarded as the world s best airport by the Airports Council International.