The Replisome Mediates A-NHEJ Repair of Telomeres Lacking POT1-TPP1 Independently of MRN Function
Summary: Telomeres use shelterin to protect chromosome ends from activating the DNA damage sensor MRE11-RAD50-NBS1 (MRN), repressing ataxia-telangiectasia, mutated (ATM) and ATM and Rad3-related (ATR) dependent DNA damage checkpoint responses.
Rekha Rai +5 more
doaj +1 more source
Effects of TPP1 overexpression on the radiosensitivity and cell cycle in HCT116 cells.
(A)Verification of TPP1 overexpression by western blotting. (B) HCT116-Mock and-TPP1 cells were irradiated with X-rays and then cell survival was determined using clonogenic assay.
Wenbo Wang (300277) +12 more
core +1 more source
Telomere Protection by TPP1 Is Mediated by POT1a and POT1b
Mammalian telomeres are protected by the shelterin complex, which contains single-stranded telomeric DNA binding proteins (POT1a and POT1b in rodents, POT1 in other mammals).
Catherine E. Keegan +3 more
core
Abstract 5745: Study on telomere shelterin component TPP1 in esophageal squamous cell carcinoma
Esophageal cancer (EC) is one of the most aggressive cancer and ranks the sixth leading cause of cancer-related mortality worldwide, approximately 70% of global esophageal carcinoma cases occur in China and esophageal squamous cell carcinoma (ESCC) is ...
Jiajing Cai +8 more
core +1 more source
Mutation update: Review of TPP1 gene variants associated with neuronal ceroid lipofuscinosis CLN2 disease [PDF]
Neuronal ceroid lipofuscinosis type 2 (CLN2 disease) is an autosomal recessive condition caused by variants in the TPP1 gene, leading to deficient activity of the lysosomal enzyme tripeptidyl peptidase I (TPP1). We update on the spectrum of TPP1 variants
Miller, N +5 more
core +1 more source
The CLN2 form of neuronal ceroid lipofuscinosis, a type of Batten disease, is a lysosomal storage disorder caused by a deficiency of the enzyme tripeptidyl peptidase-1 (TPP1).
Kennedy, Derek +17 more
core +1 more source
Combatting cellular immortality in cancers by targeting the shelterin protein complex
Shelterin proteins (TERF1, TERF2, TPP1, TINF2, POT1) protect telomeres, prevent unwarranted repair activation, and regulate telomerase activity. Alterations in these proteins can lead to cancer progression.
Sohini Chakraborty, Satarupa Banerjee
doaj +1 more source
The neuronal ceroid lipofuscinosis type 2 (CLN2) is a heterogeneous group of neurodegenerative lysosomal storage disorders caused by autosomal recessive inheritance of two pathogenic variants in trans in the TPP1 gene.
Nataliia Olkhovych +11 more
doaj +1 more source
Single-Cell and Multi-Omics-Based Characterization of Gastric Cancer Identifies TPP1 as a Potential Target for Gastric Cancer Progression and Treatment. [PDF]
Zhao Y, Ma J, Huang R, Pan S.
europepmc +1 more source
PPARα and RXRα in the regulation of neuronal ceroid lipofuscinosis genes: implications for Batten disease therapy. [PDF]
Chandra S, Pahan K.
europepmc +1 more source

