alpha 1-antitrypsin deficiency

(redirected from Alpha-1 proteinase inhibitor)

alpha 1-antitrypsin deficiency

A disorder caused most frequently by a mutation in the SERPINA 1 gene, causing the Z-allele with a substitution of lysine for glutamic acid at position 342. Alpha 1-antitrypsin is mainly produced in the liver and is protective against the proteolytic enzyme neutrophil elastase that can damage the air sacs in the lungs and the cells of the liver. People with alpha 1-antitrypsin deficiency are more prone than normal to EMPHYSEMA and to certain forms of liver disease. It is responsible for 25 per cent of deaths from juvenile liver cirrhosis. A strong case has been made that this was the disease from which the composer Frédéric Chopin died.
References in periodicals archive ?
SMC chairman Professor Jonathan Fox said: "We were unable to accept human alpha-1 proteinase inhibitor for severe emphysema we were not satisfied evidence on its long-term benefits would justify the cost.
The principal components of the global blood plasma market are immunoglobulin, albumin, factor VIII and Alpha-1 proteinase inhibitor (A1PI) or alpha-1 antitrypsin (AAT).
It is a highly purified Alpha-1 protein derived from human plasma and is reportedly the only Alpha-1 proteinase inhibitor that has been proven in a prospective double blind, placebo controlled trial (the RAPID study) to significantly reduce the loss of lung tissue, slowing the progression of emphysema due to Alpha-1 antitrypsin deficiency.
Up to 40,000 people in the United States have a genetic deficiency of the protein, alpha-1 proteinase inhibitor, also known as alpha-1 antitrypsin (SN: 5/2/87, p.
Zemaira, a human-derived alpha-1 proteinase inhibitor, acts to inhibit the activity of another protein, neutrophil elastase, which, left unchecked, can degrade the lining of the lungs and lead to pulmonary disease.
This patent is co-owned by Kamada and Pari Pharma, the producers of the eFlow nebuliser system, and covers claims regarding the unique combination of Kamada's inhaled alpha-1 proteinase inhibitor with a customized eFlow nebulizer system.
As a result of this limited distribution agreement, BioRx will also be added as a distributor of ARALAST NP, a similar product to Baxter's first alpha-1 proteinase inhibitor (A1-Pi), ARALAST, originally launched in 2003.
Endogenous human neutrophil elastase is predominantly neutralized by alpha-1 antitrypsin (AAT), also called alpha-1 proteinase inhibitor (alpha1-PI).
Prolastin is indicated for chronic augmentation therapy of individuals having congenital deficiency of alpha-1 proteinase inhibitor with clinically demonstrable panacinar emphysema.
Prolastin([R]) is indicated for chronic augmentation therapy of individuals having congenital deficiency of alpha-1 proteinase inhibitor with clinically demonstrable panacinar emphysema.
The aim of aerosolized alpha-1 proteinase inhibitor is to achieve homogeneous drug deposition within the periphery of the lungs," said Steve Petteway, Ph.
Prolastin(R) is indicated for chronic replacement therapy of individuals having congenital deficiency of alpha-1 proteinase inhibitor with clinically demonstrable panacinar emphysema.

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