Catalog No.
EHA29601
Expression system
Mammalian Cells
Species
Homo sapiens (Human)
Protein length
Met1-Pro563
Predicted molecular weight
62.76 kDa
Nature
Recombinant
Endotoxin level
Please contact with the lab for this information.
Purity
>90% as determined by SDS-PAGE.
Accession
O14773
Applications
ELISA, Immunogen, SDS-PAGE, WB, Bioactivity testing in progress
Form
Lyophilized
Storage buffer
Lyophilized from a solution in PBS pH 7.4, 1mM EDTA, 4% Trehalose, 1% Mannitol.
Reconstitution
Reconstitute in sterile water for a stock solution.A copy of datasheet will be provided with the products, please refer to it for details.
Shipping
In general, proteins are provided as lyophilized powder/frozen liquid. They are shipped out with dry ice/blue ice unless customers require otherwise.
Stability and Storage
Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt.
Alternative Names
Cell growth-inhibiting gene 1 protein, LPIC, Tripeptidyl-peptidase 1, Tripeptidyl aminopeptidase, Lysosomal pepstatin-insensitive protease, TPP1, CLN2, TPP-I, TPP-1, Tripeptidyl-peptidase I
Benchmarking Nanopore Sequencing for CLN2 (TPP1) Mutation Detection: Integrating Rapid Genomics and Orthogonal Validation for Precision Diagnostics., PMID:40507848
An AAV variant selected through NHP screens robustly transduces the brain and drives secreted protein expression in NHPs and mice., PMID:40367194
Evidence of the impact of CLN2 and CLN3 Batten disease on families in the United Kingdom., PMID:40355884
Cerebrospinal fluid proteome profiling across the Alzheimer's disease continuum: a step towards solving the equation for 'X'., PMID:40329321
Identification of Endoplasmic Reticulum Stress-Related Genes in Acute Myocardial Infarction: A Bioinformatics Approach with Experimental Validation., PMID:40319218
Novel surgical approach for intraventricular cerliponase alfa enzyme replacement therapy via central venous access device (CVAD) port in neuronal ceroid lipofuscinosis type 2 (CLN2) disease., PMID:40295329
The Impact of Dietary Melatonin on Heart and Lung Telomere Length and Shelterin Protein Gene Expression of Pulmonary Hypertensive Broiler Chickens., PMID:40257417
Mai-wei-yang-fei decoction protects against pulmonary fibrosis by reducing telomere shortening and inhibiting AECII senescence via FBW7/TPP1 regulation., PMID:40215816
Genetic Reasons for Phenotypic Diversity in Neuronal Ceroid Lipofuscinoses and High-Resolution Imaging as a Marker of Retinal Disease., PMID:40206986
C1Q+ TPP1+ macrophages promote colon cancer progression through SETD8-driven p53 methylation., PMID:40165182
Enzyme Replacement Therapy in CLN2-Associated Retinopathy., PMID:40127655
Mendelian randomization identifies proteins involved in neurodegenerative diseases., PMID:40037332
The canonical RPA complex interacts with Est3 to regulate yeast telomerase activity., PMID:39913192
Gene therapy ameliorates bowel dysmotility and enteric neuron degeneration and extends survival in lysosomal storage disorder mouse models., PMID:39813314
Intravitreal Enzyme Replacement Therapy Slows Retinopathy in Late Infantile Ceroid Lipofuscinosis Type 2., PMID:39776429
TRF1 and TRF2 form distinct shelterin subcomplexes at telomeres., PMID:39763972
Two-year follow-up of gait and postural control following initiation of recombinant human tripeptidyl intracerebroventricular enzyme replacement therapy in two atypical CLN2 patients., PMID:39762319
Twenty-Year Survival Analysis of Adeno-Associated Virus Vector Serotype 2-Mediated Gene Therapy to the Central Nervous System for CLN2 Disease., PMID:39745261
Pulmonary exposure to renewable diesel exhaust particles alters protein expression and toxicity profiles in bronchoalveolar lavage fluid and plasma of mice., PMID:39739031
Clinical, Pathological, and Molecular Findings in a Mexican Patient With Neuronal Ceroid Lipofuscinosis Type 2: Support for Pathogenicity of the c.1226 G>T Variant and for Presence of Cherry-Red Spot in This Disease., PMID:39716776
Genetic spectrum of neuronal ceroid lipofuscinosis & its genotype-phenotype correlation -A single centre experience of 56 cases., PMID:39675099
Adverse Reactions to the Orphan Drug Cerliponase Alfa in the Treatment of Neurolipofuscinosis Type 2 (CLN2)., PMID:39598424
Combatting cellular immortality in cancers by targeting the shelterin protein complex., PMID:39578854
Phenotypic variability observed in a Chinese patient cohort with biallelic variants in the CLN genes., PMID:39563673
The expression of shelterin genes and telomere repeat analysis and their effect on Alzheimer's disease., PMID:39503976
Cerliponase alfa decreases Aβ load and alters autophagy- related pathways in mouse hippocampal neurons exposed to fAβ1-42., PMID:39362589
Enzyme Replacement Therapy for CLN2 Disease: MRI Volumetry Shows Significantly Slower Volume Loss Compared with a Natural History Cohort., PMID:38977290
High Prevalence of Movement Disorder in Treated CLN2-Batten Disease: Rare Disease Therapy Development Must Not Stop With Approved Treatment., PMID:38976806
Assaying Lysosomal Enzyme Activity in Dictyostelium discoideum., PMID:38954197
Phenotypic/Genotypic Profile of Children with Neuronal Ceroid Lipofuscinosis in Southern Brazil., PMID:38857616
POT1 recruits and regulates CST-Polα/primase at human telomeres., PMID:38838667
Exploring concurrent validity of the CLN2 Clinical Rating Scale: Comparison to PedsQL using cerliponase alfa clinical trial data., PMID:38776275
A needle in a haystack? The impact of a targeted epilepsy gene panel in the identification of a treatable but rapidly progressive metabolic epilepsy: CLN2 disease., PMID:38763144
A current view of mitochondria damage and the diversity of lipopigment inclusions in neuronal ceroid lipofuscinose type 2 from rectal biopsy., PMID:38741434
TPP1 is associated with risk of advanced precursors and cervical cancer survival., PMID:38722833
Ceroid lipofuscinosis type 2 disease: Effective presymptomatic therapy-Oldest case of a presymptomatic enzyme therapy., PMID:38693756
Integrative analysis of proteomics and lipidomic profiles reveal the fat deposition and meat quality in Duroc × Guangdong small spotted pig., PMID:38659450
Clinical and Molecular Characteristics of Neuronal Ceroid Lipofuscinosis in Saudi Arabia., PMID:38653183
GABAergic interneurons contribute to the fatal seizure phenotype of CLN2 disease mice., PMID:38585903
Corrigendum to "Upregulation of tripeptidyl-peptidase 1 by 3-hydroxy-(2,2)-dimethyl butyrate, a brain endogenous ligand of PPARα: Implications for late-infantile Batten disease therapy" [Neurobiology of Disease 127 (2019) 362-373]., PMID:38548486
Among-population variation in telomere regulatory proteins and their potential role as hidden drivers of intraspecific variation in life history., PMID:38509838
Upregulation of shelterin and CST genes and longer telomeres are associated with unfavorable prognostic characteristics in prostate cancer., PMID:38503134
Cellular Modeling of CLN6 with IPSC-derived Neurons and Glia., PMID:38352418
Club cell-specific telomere protection protein 1 (TPP1) protects against tobacco smoke-induced lung inflammation, xenobiotic metabolic dysregulation, and injurious responses., PMID:38344410
Peripheral retinal finding on fluorescein angiography in neuronal ceroid lipofuscinosis type 2 (CLN2)., PMID:38341082
A computational approach to analyzing the functional and structural impacts of Tripeptidyl-Peptidase 1 missense mutations in neuronal ceroid lipofuscinosis., PMID:38185715
Characterization of novel mutations in the TEL-patch domain of the telomeric factor TPP1 associated with telomere biology disorders., PMID:38176734
Intravitreal enzyme replacement for inherited retinal diseases., PMID:38170785
For research use only. Not for human or drug use.
