EC Number   |
Natural Substrates |
|---|
   3.4.14.9 | amyloid-beta + H2O |
AbetaCy3 peptides are released from the nanofibrils due to TPP1 activity |
| 3.4.14.9 | more |
an inherited deficiency of tripeptidyl peptidase I activity causes a fatal lysosomal storage disorder, classic late infantile neuronal ceroid lipofuscinosis, CLN2 |
| 3.4.14.9 | more |
exopeptidase involved in intracellular (lysosomal) degradation of collagen fibrils |
| 3.4.14.9 | more |
involved in degradation of bone collagen |
| 3.4.14.9 | more |
classical late infantile neuronal ceroid lipofuscinosis is an autosomal recessive disease caused by mutations in the CLN2 gene resulting in functional defects of the gene product tripeptidyl-peptidase I. This disease is associated with a progressive neurodegenerative course beginning at the age of two years with developmental stagnation, finally leading to a complete loss of motor function, vision and speech by the age of 10 years |
| 3.4.14.9 | more |
elevated enzyme activity of tripeptidyl peptidase I and other lysosomal enzymes in Sjoegren's syndrome patients may play a role in tissue damage by accelerated breakdown of glycoproteins in lysosomes |
| 3.4.14.9 | more |
TPP I is the predominant proteolytic enzyme responsible for the intracellular degradation of neuromedin B. The inability of cells from patients with late-infantile neuronal ceroid lipofuscinosis (CNL2) to degrade neuromedin B and other neuropeptides may contribute to the pathogenesis of the disease |
| 3.4.14.9 | more |
dipeptidyl-peptidase I cannot functionally compensate for the loss of tripeptidyl-peptidase I |
| 3.4.14.9 | more |
TPP1F is binding partner of Dictyostelium discoideum intracellular transmembrane protein GPHR, i.e. Golgi pH regulator. A region encompassing the DUF3735 (GPHR_N) domain of GPHR is responsible for the interaction. In TPP1F, the binding site is located in the prodomain of the protein. GPHR is present in subcellular membranous compartments reaching from endoplasmic reticulum membranes to membranes of the endo-lysosomal system |
