Details for: TPP1

Gene ID: 1200

Symbol: TPP1

Ensembl ID: ENSG00000166340

Description: tripeptidyl peptidase 1

Associated with

Cells (max top 100)

(Marker Score score is uniquely calculated using our advanced thresholding algorithms to reveal cell-specific gene markers)

  • Cell Name: Kupffer cell (CL0000091)
    Fold Change: 2.53
    Marker Score: 2527
  • Cell Name: CD14-positive, CD16-negative classical monocyte (CL0002057)
    Fold Change: 2.39
    Marker Score: 133864
  • Cell Name: lung macrophage (CL1001603)
    Fold Change: 2.3
    Marker Score: 2627
  • Cell Name: alveolar macrophage (CL0000583)
    Fold Change: 2.16
    Marker Score: 55229.5
  • Cell Name: Hofbauer cell (CL3000001)
    Fold Change: 2.1
    Marker Score: 1156
  • Cell Name: CD14-positive, CD16-positive monocyte (CL0002397)
    Fold Change: 2.08
    Marker Score: 4058
  • Cell Name: CD14-positive monocyte (CL0001054)
    Fold Change: 1.87
    Marker Score: 4646
  • Cell Name: elicited macrophage (CL0000861)
    Fold Change: 1.66
    Marker Score: 10058
  • Cell Name: alternatively activated macrophage (CL0000890)
    Fold Change: 1.59
    Marker Score: 663
  • Cell Name: blood cell (CL0000081)
    Fold Change: 1.57
    Marker Score: 18266
  • Cell Name: CD1c-positive myeloid dendritic cell (CL0002399)
    Fold Change: 1.52
    Marker Score: 3933
  • Cell Name: type A enteroendocrine cell (CL0002067)
    Fold Change: 1.51
    Marker Score: 609
  • Cell Name: non-classical monocyte (CL0000875)
    Fold Change: 1.46
    Marker Score: 3534
  • Cell Name: osteoclast (CL0000092)
    Fold Change: 1.34
    Marker Score: 669
  • Cell Name: decidual cell (CL2000002)
    Fold Change: 1.34
    Marker Score: 5772.5
  • Cell Name: conventional dendritic cell (CL0000990)
    Fold Change: 1.33
    Marker Score: 914
  • Cell Name: choroid plexus epithelial cell (CL0000706)
    Fold Change: 1.31
    Marker Score: 1203
  • Cell Name: promyelocyte (CL0000836)
    Fold Change: 1.29
    Marker Score: 637
  • Cell Name: late promyelocyte (CL0002151)
    Fold Change: 1.28
    Marker Score: 321
  • Cell Name: dendritic cell, human (CL0001056)
    Fold Change: 1.26
    Marker Score: 1286
  • Cell Name: endothelial cell of sinusoid (CL0002262)
    Fold Change: 1.25
    Marker Score: 304
  • Cell Name: dopaminergic neuron (CL0000700)
    Fold Change: 1.19
    Marker Score: 12263
  • Cell Name: megakaryocyte-erythroid progenitor cell (CL0000050)
    Fold Change: 1.18
    Marker Score: 493
  • Cell Name: classical monocyte (CL0000860)
    Fold Change: 1.17
    Marker Score: 4847
  • Cell Name: eurydendroid cell (CL0000253)
    Fold Change: 1.16
    Marker Score: 474
  • Cell Name: hematopoietic cell (CL0000988)
    Fold Change: 1.16
    Marker Score: 771.5
  • Cell Name: macrophage (CL0000235)
    Fold Change: 1.16
    Marker Score: 1290
  • Cell Name: dendritic cell (CL0000451)
    Fold Change: 1.13
    Marker Score: 775
  • Cell Name: fibroblast of breast (CL4006000)
    Fold Change: 1.12
    Marker Score: 640
  • Cell Name: epithelial cell of proximal tubule (CL0002306)
    Fold Change: 1.12
    Marker Score: 3971
  • Cell Name: keratocyte (CL0002363)
    Fold Change: 1.1
    Marker Score: 253
  • Cell Name: adventitial cell (CL0002503)
    Fold Change: 1.09
    Marker Score: 270
  • Cell Name: intermediate monocyte (CL0002393)
    Fold Change: 1.09
    Marker Score: 376
  • Cell Name: acinar cell of salivary gland (CL0002623)
    Fold Change: 1.07
    Marker Score: 2435
  • Cell Name: myelocyte (CL0002193)
    Fold Change: 1.07
    Marker Score: 461
  • Cell Name: osteoblast (CL0000062)
    Fold Change: 1.07
    Marker Score: 572.5
  • Cell Name: preosteoblast (CL0007010)
    Fold Change: 1.06
    Marker Score: 300
  • Cell Name: microglial cell (CL0000129)
    Fold Change: 1.05
    Marker Score: 1911
  • Cell Name: common dendritic progenitor (CL0001029)
    Fold Change: 1.04
    Marker Score: 359.5
  • Cell Name: granulocyte monocyte progenitor cell (CL0000557)
    Fold Change: 1.02
    Marker Score: 648
  • Cell Name: papillary tips cell (CL1000597)
    Fold Change: 1.02
    Marker Score: 206
  • Cell Name: oocyte (CL0000023)
    Fold Change: 1.02
    Marker Score: 252
  • Cell Name: enterocyte (CL0000584)
    Fold Change: 1.02
    Marker Score: 4885
  • Cell Name: astrocyte of the cerebral cortex (CL0002605)
    Fold Change: 1.02
    Marker Score: 21720.5
  • Cell Name: microfold cell of epithelium of small intestine (CL1000353)
    Fold Change: 1.01
    Marker Score: 242
  • Cell Name: type II pneumocyte (CL0002063)
    Fold Change: 1
    Marker Score: 6488.5
  • Cell Name: cerebral cortex GABAergic interneuron (CL0010011)
    Fold Change: 1
    Marker Score: 71600.5
  • Cell Name: forebrain radial glial cell (CL0013000)
    Fold Change: 0.99
    Marker Score: 47823.5
  • Cell Name: stromal cell of lamina propria of small intestine (CL0009022)
    Fold Change: 0.99
    Marker Score: 223
  • Cell Name: memory B cell (CL0000787)
    Fold Change: 0.99
    Marker Score: 750
  • Cell Name: absorptive cell (CL0000212)
    Fold Change: 0.98
    Marker Score: 30372
  • Cell Name: epithelial cell of lung (CL0000082)
    Fold Change: 0.98
    Marker Score: 5140
  • Cell Name: monocyte (CL0000576)
    Fold Change: 0.98
    Marker Score: 1269
  • Cell Name: neutrophil (CL0000775)
    Fold Change: 0.97
    Marker Score: 592
  • Cell Name: retinal blood vessel endothelial cell (CL0002585)
    Fold Change: 0.97
    Marker Score: 233
  • Cell Name: cardiac mesenchymal cell (CL0000569)
    Fold Change: 0.95
    Marker Score: 235
  • Cell Name: myeloid leukocyte (CL0000766)
    Fold Change: 0.95
    Marker Score: 1148
  • Cell Name: B-1 B cell (CL0000819)
    Fold Change: 0.95
    Marker Score: 1655
  • Cell Name: bladder urothelial cell (CL1001428)
    Fold Change: 0.94
    Marker Score: 1384
  • Cell Name: mature T cell (CL0002419)
    Fold Change: 0.94
    Marker Score: 9296
  • Cell Name: intestinal crypt stem cell of colon (CL0009043)
    Fold Change: 0.94
    Marker Score: 2381
  • Cell Name: bronchial epithelial cell (CL0002328)
    Fold Change: 0.93
    Marker Score: 247
  • Cell Name: kidney loop of Henle thick ascending limb epithelial cell (CL1001106)
    Fold Change: 0.93
    Marker Score: 2496.5
  • Cell Name: early pro-B cell (CL0002046)
    Fold Change: 0.93
    Marker Score: 9421
  • Cell Name: adipocyte of epicardial fat of left ventricle (CL1000311)
    Fold Change: 0.92
    Marker Score: 243
  • Cell Name: L5 extratelencephalic projecting glutamatergic cortical neuron (CL4023041)
    Fold Change: 0.92
    Marker Score: 1421
  • Cell Name: tuft cell of colon (CL0009041)
    Fold Change: 0.92
    Marker Score: 474
  • Cell Name: bronchus fibroblast of lung (CL2000093)
    Fold Change: 0.92
    Marker Score: 1263
  • Cell Name: CD34-positive, CD38-negative hematopoietic stem cell (CL0001024)
    Fold Change: 0.92
    Marker Score: 664
  • Cell Name: malignant cell (CL0001064)
    Fold Change: 0.92
    Marker Score: 12319
  • Cell Name: smooth muscle cell of small intestine (CL1000275)
    Fold Change: 0.92
    Marker Score: 240
  • Cell Name: fibroblast of lung (CL0002553)
    Fold Change: 0.92
    Marker Score: 2399
  • Cell Name: epithelial cell of uterus (CL0002149)
    Fold Change: 0.92
    Marker Score: 210
  • Cell Name: BEST4+ intestinal epithelial cell, human (CL4030026)
    Fold Change: 0.91
    Marker Score: 431
  • Cell Name: muscle precursor cell (CL0000680)
    Fold Change: 0.91
    Marker Score: 235
  • Cell Name: extravillous trophoblast (CL0008036)
    Fold Change: 0.91
    Marker Score: 809
  • Cell Name: abnormal cell (CL0001061)
    Fold Change: 0.91
    Marker Score: 2715
  • Cell Name: pancreatic ductal cell (CL0002079)
    Fold Change: 0.91
    Marker Score: 941
  • Cell Name: luminal cell of prostate epithelium (CL0002340)
    Fold Change: 0.9
    Marker Score: 525
  • Cell Name: naive B cell (CL0000788)
    Fold Change: 0.9
    Marker Score: 804
  • Cell Name: starburst amacrine cell (CL0004232)
    Fold Change: 0.9
    Marker Score: 255
  • Cell Name: kidney distal convoluted tubule epithelial cell (CL1000849)
    Fold Change: 0.9
    Marker Score: 950
  • Cell Name: NKp44-negative group 3 innate lymphoid cell, human (CL0001080)
    Fold Change: 0.9
    Marker Score: 197
  • Cell Name: endothelial cell (CL0000115)
    Fold Change: 0.89
    Marker Score: 798
  • Cell Name: Schwann cell precursor (CL0002375)
    Fold Change: 0.89
    Marker Score: 221
  • Cell Name: intestinal epithelial cell (CL0002563)
    Fold Change: 0.89
    Marker Score: 1442
  • Cell Name: transitional stage B cell (CL0000818)
    Fold Change: 0.89
    Marker Score: 314
  • Cell Name: kidney loop of Henle thin ascending limb epithelial cell (CL1001107)
    Fold Change: 0.88
    Marker Score: 895
  • Cell Name: visceromotor neuron (CL0005025)
    Fold Change: 0.88
    Marker Score: 257
  • Cell Name: CD4-positive helper T cell (CL0000492)
    Fold Change: 0.88
    Marker Score: 952.5
  • Cell Name: leukocyte (CL0000738)
    Fold Change: 0.87
    Marker Score: 502
  • Cell Name: placental villous trophoblast (CL2000060)
    Fold Change: 0.87
    Marker Score: 3436.5
  • Cell Name: hematopoietic stem cell (CL0000037)
    Fold Change: 0.87
    Marker Score: 460
  • Cell Name: transit amplifying cell (CL0009010)
    Fold Change: 0.87
    Marker Score: 4981
  • Cell Name: stromal cell of ovary (CL0002132)
    Fold Change: 0.87
    Marker Score: 9821
  • Cell Name: neoplastic cell (CL0001063)
    Fold Change: 0.87
    Marker Score: 5285
  • Cell Name: promonocyte (CL0000559)
    Fold Change: 0.87
    Marker Score: 561
  • Cell Name: hematopoietic multipotent progenitor cell (CL0000837)
    Fold Change: 0.87
    Marker Score: 424
  • Cell Name: enterocyte of colon (CL1000347)
    Fold Change: 0.87
    Marker Score: 1338
  • Cell Name: mature gamma-delta T cell (CL0000800)
    Fold Change: 0.87
    Marker Score: 2736

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Other Information

**Key characteristics:** * TPP1 is a transmembrane protein with a molecular weight of approximately 120 kDa. * It is localized to the cell surface and is associated with various cell types. * TPP1 is a highly conserved protein with sequences found in mammals, birds, and fish. * It is a member of the peptidase family of enzymes. **Pathways and functions:** * TPP1 is involved in the regulation of inflammation and cell death. * It is a key regulator of the unfolded protein response (uPR), a cellular pathway that is triggered by stress conditions. * TPP1 is also involved in the regulation of cell migration, proliferation, and differentiation. * It is a regulator of macrophage function, including phagocytosis, migration, and activation. * TPP1 is involved in the regulation of blood cell trafficking. **Clinical significance:** * Mutations in TPP1 have been linked to several human diseases, including cancer, cardiovascular disease, and metabolic disorders. * Inhibitors of TPP1 have been investigated as potential therapeutic agents for these diseases. * Studies of TPP1 have also been used to develop new diagnostic tools for cancer and other diseases. **Additional notes:** * TPP1 is a highly regulated protein that is controlled by various factors, including growth factors, cytokines, and stress signals. * It is a key regulator of inflammation and cell death, and its activity is often increased in diseases such as cancer and autoimmune disorders. * TPP1 is a promising target for the treatment of human diseases.

Genular Protein ID: 1332768125

Symbol: TPP1_HUMAN

Name: Tripeptidyl-peptidase 1

UniProtKB Accession Codes:

O14773 Q53HT1 Q5JAK6 Q6UX56 Q71JP6 Q96C37

Database IDs:

AGR 1 AlphaFoldDB 1 Antibodypedia 1 BRENDA 1 Bgee 1 BioGRID 1 BioGRID-ORCS 1 BioMuta 1 CCDS 1 CDD 2 CTD 1 ChEBI 6 ChiTaRS 1 DIP 1 DNASU 1 DisGeNET 1 EC 1 EMBL 7 EPD 1 Ensembl 5 EvolutionaryTrace 1 ExpressionAtlas 1 GO 28 Gene3D 1 GeneCards 1 GeneTree 1 GeneWiki 1 Genevisible 1 GenomeRNAi 1 GlyConnect 1 GlyCosmos 1 GlyGen 1 HGNC 1 HOGENOM 1 HPA 1 InParanoid 1 IntAct 1 InterPro 4 KEGG 1 MANE-Select 1 MEROPS 1 MIM 5 MINT 1 MalaCards 1 MassIVE 1 MaxQB 1 NCBI Taxonomy 1 OMA 1 OpenTargets 1 Orphanet 6 OrthoDB 1 PANTHER 2 PDB 4 PDBsum 2 PRO 1 PROSITE 1 PathwayCommons 1 PaxDb 1 PeptideAtlas 1 Pfam 2 PharmGKB 1 Pharos 1 PhosphoSitePlus 1 PhylomeDB 1 Proteomes 1 ProteomicsDB 2 Pumba 1 RNAct 1 Reactome 1 RefSeq 1 SABIO-RK 1 SIGNOR 1 SMART 1 SMR 1 STRING 1 SUPFAM 2 SignaLink 1 TreeFam 1 UCSC 1 VEuPathDB 1 eggNOG 1 iPTMnet 1 jPOST 1 neXtProt 1

Citations:

PubMed ID: 9295267

Title: Association of mutations in a lysosomal protein with classical late-infantile neuronal ceroid lipofuscinosis.

PubMed ID: 9295267

DOI: 10.1126/science.277.5333.1802

PubMed ID: 9653647

Title: Structural organization and sequence of CLN2, the defective gene in classical late infantile neuronal ceroid lipofuscinosis.

PubMed ID: 9653647

DOI: 10.1006/geno.1998.5328

PubMed ID: 12975309

Title: The secreted protein discovery initiative (SPDI), a large-scale effort to identify novel human secreted and transmembrane proteins: a bioinformatics assessment.

PubMed ID: 12975309

DOI: 10.1101/gr.1293003

PubMed ID: 15489334

Title: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).

PubMed ID: 15489334

DOI: 10.1101/gr.2596504

PubMed ID: 11054422

Title: The human CLN2 protein/tripeptidyl-peptidase I is a serine protease that autoactivates at acidic pH.

PubMed ID: 11054422

DOI: 10.1074/jbc.m008562200

PubMed ID: 12643545

Title: Proteomic analysis of early melanosomes: identification of novel melanosomal proteins.

PubMed ID: 12643545

DOI: 10.1021/pr025562r

PubMed ID: 12754519

Title: Identification and quantification of N-linked glycoproteins using hydrazide chemistry, stable isotope labeling and mass spectrometry.

PubMed ID: 12754519

DOI: 10.1038/nbt827

PubMed ID: 10477428

Title: Molecular basis of the neuronal ceroid lipofuscinoses: mutations in CLN1, CLN2, CLN3, and CLN5.

PubMed ID: 10477428

DOI: 10.1002/(sici)1098-1004(1999)14:3<199::aid-humu3>3.0.co;2-a

PubMed ID: 17081065

Title: Proteomic and bioinformatic characterization of the biogenesis and function of melanosomes.

PubMed ID: 17081065

DOI: 10.1021/pr060363j

PubMed ID: 17237713

Title: Neuronal ceroid lipofuscinosis: a common pathway?

PubMed ID: 17237713

DOI: 10.1203/pdr.0b013e31802d8a4a

PubMed ID: 19941651

Title: Novel interactions of CLN5 support molecular networking between neuronal ceroid lipofuscinosis proteins.

PubMed ID: 19941651

DOI: 10.1186/1471-2121-10-83

PubMed ID: 19159218

Title: Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry.

PubMed ID: 19159218

DOI: 10.1021/pr8008012

PubMed ID: 21269460

Title: Initial characterization of the human central proteome.

PubMed ID: 21269460

DOI: 10.1186/1752-0509-5-17

PubMed ID: 24275569

Title: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.

PubMed ID: 24275569

DOI: 10.1016/j.jprot.2013.11.014

PubMed ID: 25944712

Title: N-terminome analysis of the human mitochondrial proteome.

PubMed ID: 25944712

DOI: 10.1002/pmic.201400617

PubMed ID: 19038966

Title: Structure of tripeptidyl-peptidase I provides insight into the molecular basis of late infantile neuronal ceroid lipofuscinosis.

PubMed ID: 19038966

DOI: 10.1074/jbc.m806947200

PubMed ID: 19038967

Title: Crystal structure and autoactivation pathway of the precursor form of human tripeptidyl-peptidase 1, the enzyme deficient in late infantile ceroid lipofuscinosis.

PubMed ID: 19038967

DOI: 10.1074/jbc.m806943200

PubMed ID: 10330339

Title: Mutational analysis of the defective protease in classic late-infantile neuronal ceroid lipofuscinosis, a neurodegenerative lysosomal storage disorder.

PubMed ID: 10330339

DOI: 10.1086/302427

PubMed ID: 10665500

Title: Prenatal testing for late infantile neuronal ceroid lipofuscinosis.

PubMed ID: 10665500

DOI: 10.1002/1531-8249(200002)47:2<254::aid-ana19>3.3.co;2-z

PubMed ID: 11339651

Title: Heterogeneity of late-infantile neuronal ceroid lipofuscinosis.

PubMed ID: 11339651

DOI: 10.1097/00125817-200011000-00002

PubMed ID: 11241479

Title: Two novel CLN2 gene mutations in a Chinese patient with classical late-infantile neuronal ceroid lipofuscinosis.

PubMed ID: 11241479

DOI: 10.1002/1096-8628(2001)9999:9999<::aid-ajmg1145>3.0.co;2-z

PubMed ID: 11589012

Title: New mutations in the neuronal ceroid lipofuscinosis genes.

PubMed ID: 11589012

DOI: 10.1053/ejpn.2000.0427

PubMed ID: 11462245

Title: Expression and analysis of CLN2 variants in CHO cells: Q100R represents a polymorphism, and G389E and R447H represent loss-of-function mutations.

PubMed ID: 11462245

DOI: 10.1002/humu.1170

PubMed ID: 12376936

Title: Late infantile neuronal ceroid lipofuscinosis: quantitative description of the clinical course in patients with CLN2 mutations.

PubMed ID: 12376936

DOI: 10.1002/ajmg.10660

PubMed ID: 12414822

Title: Identification of novel CLN2 mutations shows Canadian specific NCL2 alleles.

PubMed ID: 12414822

DOI: 10.1136/jmg.39.11.822

PubMed ID: 12698559

Title: Tripeptidyl peptidase 1 deficiency in neuronal ceroid lipofuscinosis. A novel mutation.

PubMed ID: 12698559

PubMed ID: 14736728

Title: Mutation of the glycosylated asparagine residue 286 in human CLN2 protein results in loss of enzymatic activity.

PubMed ID: 14736728

DOI: 10.1093/glycob/cwh054

PubMed ID: 19201763

Title: Mutations in CLN7/MFSD8 are a common cause of variant late-infantile neuronal ceroid lipofuscinosis.

PubMed ID: 19201763

DOI: 10.1093/brain/awn366

PubMed ID: 20340139

Title: Functional consequences and rescue potential of pathogenic missense mutations in tripeptidyl peptidase I.

PubMed ID: 20340139

DOI: 10.1002/humu.21251

PubMed ID: 22612257

Title: Targeted next generation sequencing as a diagnostic tool in epileptic disorders.

PubMed ID: 22612257

DOI: 10.1111/j.1528-1167.2012.03516.x

PubMed ID: 21990111

Title: Update of the mutation spectrum and clinical correlations of over 360 mutations in eight genes that underlie the neuronal ceroid lipofuscinoses.

PubMed ID: 21990111

DOI: 10.1002/humu.21624

PubMed ID: 23418007

Title: Autosomal recessive spinocerebellar ataxia 7 (SCAR7) is caused by variants in TPP1, the gene involved in classic late-infantile neuronal ceroid lipofuscinosis 2 disease (CLN2 disease).

PubMed ID: 23418007

DOI: 10.1002/humu.22292

Sequence Information:

  • Length: 563
  • Mass: 61248
  • Checksum: 7299D902F6AE8555
  • Sequence:
    • MGLQACLLGL FALILSGKCS YSPEPDQRRT LPPGWVSLGR ADPEEELSLT FALRQQNVER 
LSELVQAVSD PSSPQYGKYL TLENVADLVR PSPLTLHTVQ KWLLAAGAQK CHSVITQDFL 
TCWLSIRQAE LLLPGAEFHH YVGGPTETHV VRSPHPYQLP QALAPHVDFV GGLHRFPPTS 
SLRQRPEPQV TGTVGLHLGV TPSVIRKRYN LTSQDVGSGT SNNSQACAQF LEQYFHDSDL 
AQFMRLFGGN FAHQASVARV VGQQGRGRAG IEASLDVQYL MSAGANISTW VYSSPGRHEG 
QEPFLQWLML LSNESALPHV HTVSYGDDED SLSSAYIQRV NTELMKAAAR GLTLLFASGD 
SGAGCWSVSG RHQFRPTFPA SSPYVTTVGG TSFQEPFLIT NEIVDYISGG GFSNVFPRPS 
YQEEAVTKFL SSSPHLPPSS YFNASGRAYP DVAALSDGYW VVSNRVPIPW VSGTSASTPV 
FGGILSLINE HRILSGRPPL GFLNPRLYQQ HGAGLFDVTR GCHESCLDEE VEGQGFCSGP 
GWDPVTGWGT PNFPALLKTL LNP

Database document:

This is a preview of the gene's schema. Only a few entries are kept for 'singleCellExpressions,' 'mRNAExpressions,' and other large data arrays for visualization purposes. For the full schema, download it here.