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Alpha AntiTripsin

Hey people, help would be appreciated :biggrin:

I know the effectiveness of AAT is being studied blah blah..

But in my notes I have it down as treating Cystic Fribrosis and Emphysema. I understand how it treats Emphysema - by inhibiting Elastase - but what does it have to do with CF? I cant find anything explaining it and I cant think why it should since CF is due to a malfunction of Cl- Ion pumps and the permeability of the membrane.....:confused: :confused:

help please :biggrin:

Thanks people
Reply 1
Avatar for Fluffy
Fluffy
15
Oh! I read something on this a few weeks back - alpha1 antitrypsin is being investigated to the context of CF as a disease modifier... They *think* that the pathology involved in CF has a cascade effect and disturbs the normal protective balance the lung ordinarily exhibits to protect it from the effects of proteases and antiproteases. alpha-1 antitrypsin is the most abundant antiprotinase in the lung, so this is the one the pharms have targeted.

HHT


Edit: have just found this article, which should help: http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6WP5-46YKH5Y-9&_coverDate=12%2F31%2F2001&_alid=510337821&_rdoc=1&_fmt=&_orig=search&_qd=1&_cdi=6981&_sort=d&view=c&_acct=C000010240&_version=1&_urlVersion=0&_userid=4619704&md5=ff01968329d79b603a0c1c5db7e5bf6a
Reply 2
Avatar for Wangers
Wangers
OP
16
ok reading this: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6187052&dopt=Abstract

I think what its saying is that disease or infection (indirectly) causes inhibition of antiproteases and allows the Elastase class of enzymes to destroy the cells infected? and I see how this could cause Emphysema , and possibly CF - if the cells controlling the protein channels are destroyed by protease. Or if CF starts the cascade its a self destructive circle leading to possible other complications.

bronchoalveolar lavage - something in the alveloi?

Hmm, - thats a really interesting article - with interleukins being involved, and each of them possibly causeing a cascade and pleiotropic - even more fun:p:

Thanks Fluffy, I'll ask my parents when they get in as well.















wondering why I know about ILs? - well I was bored one lesson and fell asleep - understading some of the immune cascade stuff was the punishment :eek: It was the most intresting use of lesson time all year.
Reply 3
Avatar for Wangers
Wangers
OP
16
Thanks Fluffy :biggrin:

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