[yagoy]

Hello everyone,

I have a question I hope someone can answer it.

I understand that A1AT deficiency is due to misfolding of the mutated protein and it accumulates in the liver leading to cirrhosis.

BUT how is this misfolded, mutated protein damaging to the liver? what is the pathology that leads to this liver cirrhosis?

Thanks.

 

[elMuchacho]

accumulation of the wrong things in the wrong place always leads to pathology . so that misfolded proteins shouldn't be there , but they are still in the liver . and therefore it makes a cirrhosis . dont make things harder , you must not know how does the misfolded protein damage liver cells