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Normal Function The TPP1 gene provides instructions for making an enzyme called tripeptidyl peptidase 1. This enzyme is produced as an inactive enzyme, called a proenzyme, which has an extra segment attached. This segment must be removed, followed by additional processing steps, for the enzyme to become active.
A Tenant Protection Plan (TPP) outlines what steps the contractor and your building owner will take to protect tenants when construction or renovation happens while tenants are living in the building. The TPP works to avoid or limit service disruptions and lessen the negative impacts of construction.
CLN2 is tripeptidyl-peptidase 1 (TPP1), a serine protease that cleaves tripeptides from the amino terminus of small proteins before these tripeptides are degraded by other lysosomal proteases.
The deficiency of tripeptidyl peptidase 1 (TPP1) activity leads to accumulation of autofluorescent storage material (AFSM), which is composed of different lipids and proteins [4]. AFSM is observed in lysosomes, which are the main cellular organelles involved in degradation of high molecular compounds.
Brineura (cerliponase alfa) injection for intraventricular use is indicated to slow the loss of ambulation in symptomatic pediatric patients 3 years of age and older with late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), also known as tripeptidyl peptidase 1 (TPP1) deficiency.
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Typically, TPP1 mutations lead to neurotic cerebrum ceroidosis type 2 (CLN2), which is characterized by ataxia, seizures, progressive motor and cognitive decline, and visual impairment. CLN2 first presents at 2 to 4.5 years of age, and often leads to death before the age of 20[3].

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