Tripeptidyl-Peptidase 1

Lysosomal Serine proteases that release N-terminal tripeptide from a polypeptide and cleave peptides between hydrophobic residues. An enzyme that in humans encoded by the TPP1 gene. It is involved in the degradation of bone collagen. Mutations in this gene are associated with the CEROID LIPOFUSCINOSIS, NEURONAL, 2; and SPINOCEREBELLAR ATAXIA, AUTOSOMAL RECESSIVE CEROID LIPOFUSCINOSIS, NEURONAL, 2; AND SPINOCEREBELLAR ATAXIA, AUTOSOMAL RECESSIVE 7.

Also Known As:

Networked: 114 relevant articles (0 outcomes, 6 trials/studies)

Bio-Agent Context: Research Results

Experts

1.	Lobel, Peter: 17 articles (01/2020 - 10/2004)
2.	Sleat, David E: 14 articles (01/2020 - 10/2004)
3.	Sohar, Istvan: 9 articles (03/2014 - 05/2002)
4.	Schulz, Angela: 8 articles (01/2021 - 01/2016)
5.	Crystal, Ronald G: 8 articles (12/2020 - 11/2004)
6.	Sondhi, Dolan: 8 articles (12/2020 - 11/2004)
7.	Hackett, Neil R: 7 articles (12/2020 - 11/2004)
8.	Specchio, Nicola: 5 articles (01/2021 - 01/2016)
9.	Kohlschütter, Alfried: 5 articles (05/2019 - 04/2004)
10.	Davidson, Beverly L: 5 articles (11/2015 - 10/2004)

Related Diseases

1.	Neuronal Ceroid-Lipofuscinoses (Neuronal Ceroid Lipofuscinosis) 08/01/2020 - "The neuronal ceroid lipofuscinosis 2 (NCL2) or classic late-infantile neuronal ceroid lipofuscinosis (LINCL) is a neurogenetic disorder caused by mutations in the TPPI gene, which codes for the lysosomal tripeptidyl peptidase 1 (TPPI) EC 3.4.14.9. " 01/01/2020 - "Neuronal ceroid lipofuscinosis type 2 or CLN2 disease is a rare, autosomal recessive, neurodegenerative lysosomal storage disorder caused by tripeptidyl peptidase 1 deficiency. " 01/01/2019 - "In humans, homozygous mutations in the TPP1 gene results in loss of tripeptidyl peptidase 1 (TPP1) enzymatic activity, leading to late infantile neuronal ceroid lipofuscinoses disease. " 01/01/2018 - "Inducible transgenic expression of tripeptidyl peptidase 1 in a mouse model of late-infantile neuronal ceroid lipofuscinosis." 03/01/2013 - "Tripeptidyl-peptidase 1 (TPP1) null or residual activity occurs in neuronal ceroid lipofuscinosis (NCL) with underlying TPP1/CLN2 mutations. "
2.	Ataxia (Dyssynergia) 11/08/2021 - "In this study, we used a well-characterized mouse model of classical late infantile NCL (cLINCL) in which the tripeptidyl peptidase 1 (Tpp1) gene is disrupted by gene targeting, resulting in loss of detectable TPP1 activity and leading to progressive neurological phenotypes including ataxia, increased motor deficiency, and early death. " 05/01/2013 - "SCAR7 patients showed ataxia and low activity of tripeptidyl-peptidase 1, but no ophthalmologic abnormalities or epilepsy. " 10/01/2022 - "CLN2 Batten disease is a lysosomal disorder in which pathogenic variants in CLN2 lead to reduced activity in the enzyme tripeptidyl peptidase 1. The disease typically manifests around 2 to 4 years of age with developmental delay, ataxia, seizures, inability to speak and walk, and fatality between 6 and 12 years of age. "
3.	Pre-Eclampsia (Preeclampsia) 01/01/2022 - "A recent proteomics study of human placental tissue has identified that tripeptidyl peptidase 1 (TPP1) production is reduced in the placenta in early-onset preeclampsia compared to uncomplicated pregnancy. "
4.	Melanoma (Melanoma, Malignant) 01/01/2023 - "A new study in Science identified somatic variants in the promoter of the gene that encodes telomere shelterin protein TPP1 in human melanomas. "
5.	Rheumatoid Arthritis 05/01/2002 - "In this study, the activities of the lysosomal cysteine proteases cathepsin B and H, dipeptidyl peptidase I, and the serine protease tripeptidyl peptidase I and dipeptidyl peptidase II, all ascribed a role in collagen digestion, were compared with those of the aspartate protease cathepsin D, and lysosomal glycosidases in leukocytes from rheumatoid arthritis patients at different stages of the disease. "

Related Drugs and Biologics

1.	Enzymes
2.	Peptide Hydrolases (Proteases)
3.	Serine Proteases (Serine Protease)
4.	Cathepsin D
5.	Cathepsin B
6.	Cathepsin C
7.	Lysergic Acid Diethylamide (LSD)
8.	Cysteine Proteases (Cysteine Protease)
9.	Glycoside Hydrolases (Endoglycosidases)
10.	Collagen

Related Therapies and Procedures

1.	Therapeutics
2.	Enzyme Replacement Therapy