In: Biology
State and Describe the location for the transcription and translation of glucocerebrosidase (Gaucher's Diease)
Please explain
Site of transcription of glucocerberosidase enzyme is nucleus and translation starts in cytosol and completed in ER.
Lysosomes are single membrane bound organelles and are derived from golgi body. About 50 digestive enzymes are present in lysosomes called acid hydrolysis as hydrolytic activity is performed at acidic pH.One of the digestive enzyme is glucocereberosidase enzyme which breaks glucocereberosides into sugar.
if this enzyme is missing, it leads to accumulation of glucocereberosides and this disease is known as Gaucher disease and this is a Autosomal recessive disorder.Main symptoms include fatigue,anemia,low platelet count,enlarged liver and spleen and if bleeding occurs it is difficult to stop.
The enzyme is 1st transcribed in nucleus and than mRNA is transferred in cytosol where translation starts and completes in ER. All proteins of lysosomes are secretory proteins.
In this way glucocereberosidase is translated and targeted to lysosome.