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Symbol CTSZ contributors: mct - updated : 11-05-2016
HGNC name cathepsin Z
HGNC id 2547
Location 20q13.32      Physical location : 57.570.241 - 57.582.309
Synonym name
  • lysosomal cysteine carboxypeptidase cathepsin X
  • preprocathepsin P
  • Synonym symbol(s) CTSX, CATX
    EC.number 3.4.22.-/
    TYPE functioning gene
    SPECIAL FEATURE opposite orientation, tail to tail
    text tail to tail with TH1l
    STRUCTURE 12.08 kb     6 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    regulatory sequence Promoter
    Binding site   transcription factor
    text structure absence of a TATA box, a high G+C content, a CpG island and the presence of several Sp1-binding sites, housekeeping gene
    MAPPING cloned Y linked   status confirmed
    Physical map
    LOC284754 20q13.31 hypothetical gene supported by BC037891 SPO11 20q13.2-q13.3 SPO11 meiotic protein covalently bound to DSB-like (S. cerevisiae) RAE1 20q13.31 RAE1 RNA export 1 homolog (S. pombe) RNPC1 20q13.31 RNA-binding region (RNP1, RRM) containing 1 LOC149767 20q13.31 similar to dJ579F20.1 (high-mobility group (nonhistone chromosomal) protein 1-like 1) CTCFL 20q13.31 CCCTC-binding factor (zinc finger protein)-like PCK1 20q13.3 phosphoenolpyruvate carboxykinase 1 (soluble) ZBP1 17q21.3 Z-DNA binding protein 1 TMEPAI 20q13.31-q13.33 transmembrane, prostate androgen induced RNA C20orf85 20q13.32 chromosome 20 open reading frame 85 C20orf86 20q13.32 chromosome 20 open reading frame 86 PPP4R1L 20q13.32 protein phosphatase 4, regulatory subunit 1-like RAB22A 20q13 RAB22A, member RAS oncogene family VAPB 20q13 VAMP (vesicle-associated membrane protein)-associated protein B and C FLJ90166 20q13.32 hypothetical protein FLJ90166 MGC4294 20q13.32 hypothetical protein MGC4294 LOC388801 20 LOC388801 STX16 20q13.32 syntaxin 16 NPEPL1 20q13.32 aminopeptidase-like 1 LOC388802 20 hypothetical gene supported by AK124059 LOC391258 20 similar to Hypothetical protein KIAA0233 GNAS 20q13.2-q13.3 GNAS complex locus TH1L 20q13 TH1-like (Drosophila) CTSZ 20q13 cathepsin Z TUBB1 20q13.32 tubulin, beta 1 ATP5E 20q13.2-q13.3 ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit C20orf45 20q13.32 chromosome 20 open reading frame 45 MRPS16P 20q13.32 chromosome 20 open reading frame 45 LOC343629 20q13.32 similar to bA379F14.2 (novel protein) C20orf174 20q13.32 chromosome 20 open reading frame 174 EDN3 20q13.2-q13.3 endothelin 3 LOC391259 20 similar to bA164D18.1 (novel protein similar to KIAA0233) SCAPIN1 20q13.32-q13.33 scapinin SYCP2 20q13.33 synaptonemal complex protein 2 PPP1R3D 20q13.3 protein phosphatase 1, regulatory subunit 3D C20orf177 20q13.2-q13.33 chromosome 20 open reading frame 177 CDH26 20q13.2-q13.33 cadherin-like 26 FLJ33860 20q13.33 hypothetical protein FLJ33860 LOC388803 20 LOC388803 LOC388804 20 LOC388804 MTCO2L 20 cytochrome c oxidase II-like
    regionally located tail to tail with TH1l
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    6 - 1517 - 303 - 2012 22451661
    Type widely
       expressed in (based on citations)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestiveintestinelarge intestinecolon highly
     stomach   highly
    Nervousbrain     Homo sapiens
    Reproductivefemale systembreastmammary gland highly
    SystemCellPubmedSpeciesStageRna symbol
    Blood/Hematopoieticmonocyte Homo sapiens
    Lymphoid/Immunemacrophage Homo sapiens
    cell lineage
    cell lines cancer cell lines
    at STAGE
  • a very short proregion
  • a three residue insertion between Gly22 and Cys31
  • a highly conserved region of the catalytic triad,
  • a second insertion homologous to the occluding loop in CTSB
  • an RGD motif located in a highly exposed region of the propeptide, which may allow binding of the proenzyme to RGD-recognizing integrins
  • isoforms Precursor
    FAMILY papain family of thiol proteases
    CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     plasma membrane
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    basic FUNCTION
  • placental cysteine proteinase, with putative exopeptidase activity
  • may be involved in tumorigenesis
  • has a restricted catalytic specificity
  • extracellular function of cathepsin Z may include binding to integrins thereby modulating the attachment of migrating cells to extracellular matrix components
  • with BLMH, are both cysteine proteases of a papain-like structure, and mediate N-terminal proteolysis and toxicity of mutant huntingtin
  • cysteine carboxypeptidase, that is involved in the regulation of T cell migration by interaction with ITGAL, ITGB2
  • potential role of cathepsin Z in bypassing cellular senescence
  • CTSL, CTSZ, account for the lysosome's capacity to digest polyQ sequences, and are important in defending against the accumulation and toxicity of polyQ proteins
  • several of the tumor-promoting functions of CTSZ were not dependent on its described catalytic activity but instead were mediated via the Arg-Gly-Asp (RGD) motif in the enzyme prodomain, which regulated interactions with integrins and the extracellular matrix
  • role for CTSZ in the anti-tumor immune response
  • predominantly supplied by tumor-associated macrophages, and is the compensatory protease that regulates the acquired tumor-promoting functions of lesions deficient in both CTSB and CTSS
  • CELLULAR PROCESS protein, degradation
    a component
    small molecule
  • interaction with ITGAL
  • it is likely that CTSL carries out initial cleavages yielding shorter peptides that are hydrolyzed to amino acids by CTSZ
  • cell & other
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --over  
    in high grade intraepithelial colorectal neoplasia and in early tumor stages
  • to tuberculosis
  • Variant & Polymorphism SNP
  • rs34069356 in the 3 prime UTR of CTSZ showed significant association with tuberculosis susceptibility
  • Candidate gene
    Therapy target
    neurologyneurodegenerativehuntington chorea
    with BLMH, are potential novel targets for Huntington disease therapeutics