Details for: CLPP

Gene ID: 8192

Symbol: CLPP

Ensembl ID: ENSG00000125656

Description: caseinolytic mitochondrial matrix peptidase proteolytic subunit

Associated with

Cells (max top 100)

(Cell Significance Index and respective Thresholds are uniquely calculated using our advanced thresholding algorithms to reveal cell-specific gene markers)

  • Cell Name: polychromatophilic erythroblast (CL0000550)
    Fold Change: 189.6233
    Cell Significance Index: -29.5000
  • Cell Name: hematopoietic oligopotent progenitor cell (CL0002032)
    Fold Change: 108.5185
    Cell Significance Index: -27.5300
  • Cell Name: embryonic stem cell (CL0002322)
    Fold Change: 92.7788
    Cell Significance Index: -38.2200
  • Cell Name: mucosal type mast cell (CL0000485)
    Fold Change: 83.6047
    Cell Significance Index: -33.9700
  • Cell Name: smooth muscle fiber of ileum (CL1000278)
    Fold Change: 80.1698
    Cell Significance Index: -37.8500
  • Cell Name: peripheral blood mononuclear cell (CL2000001)
    Fold Change: 74.8855
    Cell Significance Index: -38.5200
  • Cell Name: ileal goblet cell (CL1000326)
    Fold Change: 56.8834
    Cell Significance Index: -38.1700
  • Cell Name: ciliated cell of the bronchus (CL0002332)
    Fold Change: 35.9050
    Cell Significance Index: -34.2800
  • Cell Name: orthochromatic erythroblast (CL0000552)
    Fold Change: 28.5087
    Cell Significance Index: -35.1500
  • Cell Name: CD8-alpha-beta-positive, alpha-beta intraepithelial T cell (CL0000796)
    Fold Change: 11.2697
    Cell Significance Index: -30.1900
  • Cell Name: stromal cell of bone marrow (CL0010001)
    Fold Change: 9.2903
    Cell Significance Index: -36.6600
  • Cell Name: CD8-positive, alpha-beta regulatory T cell (CL0000795)
    Fold Change: 8.8818
    Cell Significance Index: -27.2800
  • Cell Name: CD4-positive, alpha-beta memory T cell, CD45RO-positive (CL0001204)
    Fold Change: 2.5439
    Cell Significance Index: 74.7100
  • Cell Name: microcirculation associated smooth muscle cell (CL0008035)
    Fold Change: 2.2655
    Cell Significance Index: 19.0300
  • Cell Name: fibroblast of dermis (CL0002551)
    Fold Change: 1.0670
    Cell Significance Index: 22.3400
  • Cell Name: acinar cell of salivary gland (CL0002623)
    Fold Change: 1.0632
    Cell Significance Index: 49.5700
  • Cell Name: microfold cell of epithelium of small intestine (CL1000353)
    Fold Change: 1.0018
    Cell Significance Index: 69.2800
  • Cell Name: stromal cell of ovary (CL0002132)
    Fold Change: 0.9531
    Cell Significance Index: 130.8900
  • Cell Name: tonsil germinal center B cell (CL2000006)
    Fold Change: 0.9282
    Cell Significance Index: 109.4600
  • Cell Name: early pro-B cell (CL0002046)
    Fold Change: 0.9110
    Cell Significance Index: 58.7800
  • Cell Name: conjunctival epithelial cell (CL1000432)
    Fold Change: 0.8854
    Cell Significance Index: 12.0800
  • Cell Name: cell in vitro (CL0001034)
    Fold Change: 0.7938
    Cell Significance Index: 433.5100
  • Cell Name: tuft cell of colon (CL0009041)
    Fold Change: 0.7705
    Cell Significance Index: 695.6800
  • Cell Name: basal cell of prostate epithelium (CL0002341)
    Fold Change: 0.7311
    Cell Significance Index: 19.9000
  • Cell Name: bladder urothelial cell (CL1001428)
    Fold Change: 0.7169
    Cell Significance Index: 37.2400
  • Cell Name: luminal adaptive secretory precursor cell of mammary gland (CL4033057)
    Fold Change: 0.7097
    Cell Significance Index: 33.3600
  • Cell Name: basal cell of urothelium (CL1000486)
    Fold Change: 0.6417
    Cell Significance Index: 78.9000
  • Cell Name: skeletal muscle myoblast (CL0000515)
    Fold Change: 0.6200
    Cell Significance Index: 6.7400
  • Cell Name: intermediate cell of urothelium (CL4030055)
    Fold Change: 0.6051
    Cell Significance Index: 109.0800
  • Cell Name: granulosa cell (CL0000501)
    Fold Change: 0.5503
    Cell Significance Index: 14.4700
  • Cell Name: progenitor cell of mammary luminal epithelium (CL0009116)
    Fold Change: 0.5145
    Cell Significance Index: 38.3500
  • Cell Name: hair follicular keratinocyte (CL2000092)
    Fold Change: 0.5125
    Cell Significance Index: 226.5900
  • Cell Name: sebum secreting cell (CL0000317)
    Fold Change: 0.5084
    Cell Significance Index: 35.9600
  • Cell Name: odontoblast (CL0000060)
    Fold Change: 0.4867
    Cell Significance Index: 62.3900
  • Cell Name: lactocyte (CL0002325)
    Fold Change: 0.4582
    Cell Significance Index: 59.2000
  • Cell Name: paneth cell of epithelium of small intestine (CL1000343)
    Fold Change: 0.4168
    Cell Significance Index: 9.0300
  • Cell Name: endothelial cell of venule (CL1000414)
    Fold Change: 0.3517
    Cell Significance Index: 4.0000
  • Cell Name: mesonephric nephron tubule epithelial cell (CL1000022)
    Fold Change: 0.2921
    Cell Significance Index: 10.1500
  • Cell Name: glycinergic neuron (CL1001509)
    Fold Change: 0.2729
    Cell Significance Index: 14.3300
  • Cell Name: L2/3-6 intratelencephalic projecting glutamatergic neuron (CL4023040)
    Fold Change: 0.2469
    Cell Significance Index: 49.5200
  • Cell Name: enteroendocrine cell of colon (CL0009042)
    Fold Change: 0.2384
    Cell Significance Index: 45.3700
  • Cell Name: enterocyte of epithelium of small intestine (CL1000334)
    Fold Change: 0.1982
    Cell Significance Index: 5.7100
  • Cell Name: kidney cell (CL1000497)
    Fold Change: 0.1804
    Cell Significance Index: 1.4400
  • Cell Name: epithelial cell of small intestine (CL0002254)
    Fold Change: 0.1437
    Cell Significance Index: 23.3800
  • Cell Name: obsolete caudal ganglionic eminence derived GABAergic cortical interneuron (CL4023070)
    Fold Change: 0.1429
    Cell Significance Index: 51.2500
  • Cell Name: eye photoreceptor cell (CL0000287)
    Fold Change: 0.1341
    Cell Significance Index: 8.4500
  • Cell Name: peg cell (CL4033014)
    Fold Change: 0.1078
    Cell Significance Index: 2.4900
  • Cell Name: neoplastic cell (CL0001063)
    Fold Change: 0.0909
    Cell Significance Index: 18.0300
  • Cell Name: interstitial cell of ovary (CL0002094)
    Fold Change: 0.0734
    Cell Significance Index: 0.9400
  • Cell Name: GABAergic interneuron (CL0011005)
    Fold Change: 0.0649
    Cell Significance Index: 44.9100
  • Cell Name: endothelial cell of placenta (CL0009092)
    Fold Change: 0.0513
    Cell Significance Index: 0.3100
  • Cell Name: colon goblet cell (CL0009039)
    Fold Change: 0.0466
    Cell Significance Index: 4.6100
  • Cell Name: pulmonary alveolar epithelial cell (CL0000322)
    Fold Change: 0.0405
    Cell Significance Index: 30.6500
  • Cell Name: placental villous trophoblast (CL2000060)
    Fold Change: 0.0232
    Cell Significance Index: 0.6200
  • Cell Name: mesenchymal cell (CL0008019)
    Fold Change: 0.0108
    Cell Significance Index: 0.1800
  • Cell Name: kidney loop of Henle cortical thick ascending limb epithelial cell (CL1001109)
    Fold Change: 0.0049
    Cell Significance Index: 3.6200
  • Cell Name: enterocyte of epithelium of large intestine (CL0002071)
    Fold Change: -0.0007
    Cell Significance Index: -0.0300
  • Cell Name: neutrophil progenitor cell (CL0000834)
    Fold Change: -0.0022
    Cell Significance Index: -0.0600
  • Cell Name: pancreatic acinar cell (CL0002064)
    Fold Change: -0.0065
    Cell Significance Index: -1.1100
  • Cell Name: pigmented epithelial cell (CL0000529)
    Fold Change: -0.0070
    Cell Significance Index: -13.1100
  • Cell Name: abnormal cell (CL0001061)
    Fold Change: -0.0196
    Cell Significance Index: -2.0000
  • Cell Name: anterior lens cell (CL0002223)
    Fold Change: -0.0202
    Cell Significance Index: -37.2200
  • Cell Name: pro-T cell (CL0000827)
    Fold Change: -0.0215
    Cell Significance Index: -0.5500
  • Cell Name: lens epithelial cell (CL0002224)
    Fold Change: -0.0239
    Cell Significance Index: -36.7900
  • Cell Name: secondary lens fiber (CL0002225)
    Fold Change: -0.0313
    Cell Significance Index: -42.5500
  • Cell Name: pancreatic A cell (CL0000171)
    Fold Change: -0.0421
    Cell Significance Index: -31.1900
  • Cell Name: non-pigmented ciliary epithelial cell (CL0002304)
    Fold Change: -0.0463
    Cell Significance Index: -29.4300
  • Cell Name: type B pancreatic cell (CL0000169)
    Fold Change: -0.0538
    Cell Significance Index: -30.3200
  • Cell Name: ciliary muscle cell (CL1000443)
    Fold Change: -0.0686
    Cell Significance Index: -31.1500
  • Cell Name: pancreatic PP cell (CL0002275)
    Fold Change: -0.0798
    Cell Significance Index: -49.8600
  • Cell Name: transit amplifying cell of colon (CL0009011)
    Fold Change: -0.0927
    Cell Significance Index: -2.9700
  • Cell Name: epithelial cell of stomach (CL0002178)
    Fold Change: -0.0965
    Cell Significance Index: -11.2500
  • Cell Name: dopaminergic neuron (CL0000700)
    Fold Change: -0.1084
    Cell Significance Index: -31.2000
  • Cell Name: lung endothelial cell (CL1001567)
    Fold Change: -0.1090
    Cell Significance Index: -5.6800
  • Cell Name: small intestine goblet cell (CL1000495)
    Fold Change: -0.1602
    Cell Significance Index: -5.6300
  • Cell Name: pancreatic ductal cell (CL0002079)
    Fold Change: -0.1737
    Cell Significance Index: -19.9000
  • Cell Name: pancreatic D cell (CL0000173)
    Fold Change: -0.2046
    Cell Significance Index: -43.0900
  • Cell Name: umbrella cell of urothelium (CL4030056)
    Fold Change: -0.2047
    Cell Significance Index: -1.8900
  • Cell Name: basal epithelial cell of tracheobronchial tree (CL0002329)
    Fold Change: -0.2057
    Cell Significance Index: -5.7500
  • Cell Name: pigmented ciliary epithelial cell (CL0002303)
    Fold Change: -0.2185
    Cell Significance Index: -31.7600
  • Cell Name: cortical cell of adrenal gland (CL0002097)
    Fold Change: -0.2241
    Cell Significance Index: -6.0100
  • Cell Name: paneth cell of colon (CL0009009)
    Fold Change: -0.3010
    Cell Significance Index: -4.5100
  • Cell Name: forebrain neuroblast (CL1000042)
    Fold Change: -0.3238
    Cell Significance Index: -19.9000
  • Cell Name: kidney loop of Henle descending limb epithelial cell (CL1001021)
    Fold Change: -0.3331
    Cell Significance Index: -26.3800
  • Cell Name: fibroblast of mammary gland (CL0002555)
    Fold Change: -0.3363
    Cell Significance Index: -9.6400
  • Cell Name: smooth muscle cell of sphincter of pupil (CL0002243)
    Fold Change: -0.3417
    Cell Significance Index: -35.5800
  • Cell Name: intestinal crypt stem cell of small intestine (CL0009017)
    Fold Change: -0.3662
    Cell Significance Index: -7.8000
  • Cell Name: cardiac muscle myoblast (CL0000513)
    Fold Change: -0.3787
    Cell Significance Index: -29.0600
  • Cell Name: retinal progenitor cell (CL0002672)
    Fold Change: -0.3943
    Cell Significance Index: -22.1300
  • Cell Name: enteroendocrine cell of small intestine (CL0009006)
    Fold Change: -0.4248
    Cell Significance Index: -10.6200
  • Cell Name: hippocampal granule cell (CL0001033)
    Fold Change: -0.4283
    Cell Significance Index: -28.8000
  • Cell Name: kidney epithelial cell (CL0002518)
    Fold Change: -0.4889
    Cell Significance Index: -14.4000
  • Cell Name: retinal rod cell (CL0000604)
    Fold Change: -0.5855
    Cell Significance Index: -6.9800
  • Cell Name: intestinal epithelial cell (CL0002563)
    Fold Change: -0.5989
    Cell Significance Index: -6.2000
  • Cell Name: intestinal tuft cell (CL0019032)
    Fold Change: -0.6226
    Cell Significance Index: -38.1700
  • Cell Name: CD4-positive, alpha-beta thymocyte (CL0000810)
    Fold Change: -0.6625
    Cell Significance Index: -11.4200
  • Cell Name: CD14-positive, CD16-negative classical monocyte (CL0002057)
    Fold Change: -0.6952
    Cell Significance Index: -12.8500
  • Cell Name: indirect pathway medium spiny neuron (CL4023029)
    Fold Change: -0.7121
    Cell Significance Index: -31.5000
  • Cell Name: vascular lymphangioblast (CL0005022)
    Fold Change: -0.7190
    Cell Significance Index: -12.7100
  • Cell Name: cerebellar granule cell (CL0001031)
    Fold Change: -0.7898
    Cell Significance Index: -13.5400

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this specific cell.

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this cell type. Calculated using techniques like effect size estimation and bootstrapping for reliability.

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this cell type. Calculated using techniques like effect size estimation and bootstrapping for reliability.

Other Information

**Key Characteristics:** 1. **Protein Degradation:** CLPP is a proteolytic enzyme that breaks down misfolded or incompletely synthesized proteins within the mitochondrial matrix. This function is essential for maintaining protein quality control and preventing the accumulation of aberrant proteins that can lead to cellular stress and disease. 2. **Mitochondrial Localization:** CLPP is primarily localized to the mitochondrial matrix, where it can interact with other mitochondrial proteins and regulate protein degradation. 3. **Serine-Type Endopeptidase:** CLPP possesses serine-type endopeptidase activity, which enables it to cleave peptide bonds in proteins, facilitating protein degradation. 4. **Cellular Expression:** CLPP is expressed in various cell types, including immune cells, epithelial cells, and blood cells, underscoring its importance in maintaining cellular homeostasis. **Pathways and Functions:** 1. **Atp-Dependent Peptidase Activity:** CLPP is involved in ATP-dependent peptidase activity, which is essential for regulating protein degradation and catabolic processes. 2. **Protein Binding:** CLPP interacts with other proteins, including the Clp protease complex, to regulate protein degradation and quality control. 3. **Metabolism of Proteins:** CLPP is involved in the metabolism of proteins, including the degradation of misfolded or incompletely synthesized proteins. 4. **Proteolysis Involved in Protein Catabolic Process:** CLPP plays a crucial role in proteolysis involved in protein catabolic processes, highlighting its importance in regulating protein degradation and cellular homeostasis. **Clinical Significance:** 1. **Immunological Disorders:** Dysregulation of CLPP has been implicated in various immunological disorders, including autoimmune diseases and immunodeficiency disorders. 2. **Mitochondrial Diseases:** CLPP dysfunction has been linked to mitochondrial diseases, including neurodegenerative disorders and metabolic disorders. 3. **Cancer:** CLPP expression has been observed in various types of cancer, suggesting its potential role in cancer development and progression. 4. **Protein Misfolding Diseases:** CLPP's involvement in protein quality control highlights its potential role in preventing protein misfolding diseases, such as Alzheimer's and Parkinson's disease. In conclusion, CLPP is a vital component of the mitochondrial matrix that plays a critical role in protein quality control and cellular homeostasis. Its dysregulation has been implicated in various immunological and mitochondrial disorders, highlighting the importance of this gene in maintaining overall health and preventing disease.

Genular Protein ID: 903192004

Symbol: CLPP_HUMAN

Name: ATP-dependent Clp protease proteolytic subunit, mitochondrial

UniProtKB Accession Codes:

Q16740 B2R4W5

Database IDs:

AGR 1 AlphaFoldDB 1 Antibodypedia 1 BRENDA 1 Bgee 1 BindingDB 1 BioGRID 1 BioGRID-ORCS 1 BioMuta 1 CCDS 1 CDD 1 CORUM 1 CTD 1 ChEMBL 1 ChiTaRS 1 ComplexPortal 1 DIP 1 DMDM 1 DNASU 1 DisGeNET 1 DrugBank 1 EC 1 EMBL 3 Ensembl 1 EvolutionaryTrace 1 ExpressionAtlas 1 GO 14 GeneCards 1 GeneReviews 1 GeneTree 1 GeneWiki 1 GenomeRNAi 1 GuidetoPHARMACOLOGY 1 HAMAP 1 HGNC 1 HPA 1 InParanoid 1 IntAct 1 InterPro 5 KEGG 1 MANE-Select 1 MEROPS 1 MIM 3 MINT 1 MalaCards 1 MassIVE 1 MetOSite 1 NCBI Taxonomy 1 OMA 1 OpenTargets 1 Orphanet 2 OrthoDB 1 PANTHER 2 PDB 14 PDBsum 14 PIR 1 PRINTS 1 PRO 1 PROSITE 2 PathwayCommons 1 PaxDb 1 PeptideAtlas 1 Pfam 1 PharmGKB 1 Pharos 1 PhosphoSitePlus 1 PhylomeDB 1 Proteomes 1 ProteomicsDB 1 Pumba 1 RNAct 1 Reactome 1 RefSeq 1 SMR 1 STRING 1 SUPFAM 1 SignaLink 1 SwissPalm 1 TopDownProteomics 1 TreeFam 1 UCSC 1 VEuPathDB 1 eggNOG 1 iPTMnet 1 jPOST 1 neXtProt 1

Citations:

PubMed ID: 8543061

Title: Human ClpP protease: cDNA sequence, tissue-specific expression and chromosomal assignment of the gene.

PubMed ID: 8543061

DOI: 10.1016/0014-5793(95)01353-9

PubMed ID: 14702039

Title: Complete sequencing and characterization of 21,243 full-length human cDNAs.

PubMed ID: 14702039

DOI: 10.1038/ng1285

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: 19892738

Title: Global profiling of protease cleavage sites by chemoselective labeling of protein N-termini.

PubMed ID: 19892738

DOI: 10.1073/pnas.0908958106

PubMed ID: 10525407

Title: Mitochondrial localization and oligomeric structure of HClpP, the human homologue of E. coli ClpP.

PubMed ID: 10525407

DOI: 10.1006/jmbi.1999.3121

PubMed ID: 11923310

Title: Functional proteolytic complexes of the human mitochondrial ATP-dependent protease, hClpXP.

PubMed ID: 11923310

DOI: 10.1074/jbc.m201642200

PubMed ID: 16115876

Title: Human mitochondrial ClpP is a stable heptamer that assembles into a tetradecamer in the presence of ClpX.

PubMed ID: 16115876

DOI: 10.1074/jbc.m507240200

PubMed ID: 19413330

Title: Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach.

PubMed ID: 19413330

DOI: 10.1021/ac9004309

PubMed ID: 19608861

Title: Lysine acetylation targets protein complexes and co-regulates major cellular functions.

PubMed ID: 19608861

DOI: 10.1126/science.1175371

PubMed ID: 21269460

Title: Initial characterization of the human central proteome.

PubMed ID: 21269460

DOI: 10.1186/1752-0509-5-17

PubMed ID: 22354088

Title: Mitochondrial processing peptidase regulates PINK1 processing, import and Parkin recruitment.

PubMed ID: 22354088

DOI: 10.1038/embor.2012.14

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: 15522782

Title: Crystallography and mutagenesis point to an essential role for the N-terminus of human mitochondrial ClpP.

PubMed ID: 15522782

DOI: 10.1016/j.jsb.2004.07.004

PubMed ID: 23541340

Title: Perrault syndrome is caused by recessive mutations in CLPP, encoding a mitochondrial ATP-dependent chambered protease.

PubMed ID: 23541340

DOI: 10.1016/j.ajhg.2013.02.013

PubMed ID: 25956234

Title: Exome analysis identified a novel missense mutation in the CLPP gene in a consanguineous Saudi family expanding the clinical spectrum of Perrault Syndrome type-3.

PubMed ID: 25956234

DOI: 10.1016/j.jns.2015.04.038

Sequence Information:

  • Length: 277
  • Mass: 30180
  • Checksum: B03AAC5D50F7880E
  • Sequence:
    • MWPGILVGGA RVASCRYPAL GPRLAAHFPA QRPPQRTLQN GLALQRCLHA TATRALPLIP 
IVVEQTGRGE RAYDIYSRLL RERIVCVMGP IDDSVASLVI AQLLFLQSES NKKPIHMYIN 
SPGGVVTAGL AIYDTMQYIL NPICTWCVGQ AASMGSLLLA AGTPGMRHSL PNSRIMIHQP 
SGGARGQATD IAIQAEEIMK LKKQLYNIYA KHTKQSLQVI ESAMERDRYM SPMEAQEFGI 
LDKVLVHPPQ DGEDEPTLVQ KEPVEAAPAA EPVPAST

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. You can zoom in with the mouse wheel for a closer view, and the text will adjust automatically if necessary. For the full schema, download it here.