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Baylor researchers pinpoint location of enzyme contributing to Alpha-1 liver disease
The cover story of the scientific journal Molecular Biology of the Cell shows the location in human cells of an enzyme which may be the key to rapid development of liver disease in Alpha-1 Antitrypsin Deficiency — including life-threatening liver disease in some newborns with Alpha-1.
An enzyme called ERMan1 controls the breakdown of soluble alpha-1 protein before it can begin to accumulate in the liver cell.
The enzyme ERMan1 is shown in green, around the nucleus of three cells, in this cover photo from the journal Molecular Biology of the Cell
Reproduced with permission of the American Society for Cell Biology www.ascb.org
Researchers at Baylor College of Medicine in Houston, Texas, believe that a mutation in ERMan1 may be responsible for the severity of Alpha-1-related liver disease, the most common genetic form of childhood liver disease.
In a study featured on the Aug. 15 cover of the journal, Shujuan Pan, Richard Sifers and colleagues at Baylor show that ERMan1 is concentrated in the Golgi complex surrounding the cell nucleus.
