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FLASH GENE
Symbol LTBP2 contributors: mct/npt - updated : 25-05-2018
HGNC name latent transforming growth factor beta binding protein 2
HGNC id 6715
EXPRESSION
Type widely
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Endocrinethyroid   highly
Nervousnerve   highly
Respiratorylung   highly
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Connectivebone  highly
cell lineage
cell lines melanoma
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • a N terminal fibrillin 4-cys motif
  • a non cbEGF-like motif
  • a proline-rich linker
  • a LT 4-cys motif
  • a second non cbEGF, 18 other EGF-like motifs (one each)
  • four cystine repeats (one of F, three of LT type)
  • RGD putative integrin recognition site
  • C-terminal fragment could block the interaction of the LTBP1 fragment with the fibrillin (Hirani 2007)
  • mono polymer homomer
    HOMOLOGY
    intraspecies homolog to LTBP1
    homolog to fibrillin
    Homologene
    FAMILY
  • fibrillin/LTBP extracellular matrix glycoprotein superfamily
  • LTBP family
  • CATEGORY adhesion , structural protein , signaling growth factor
    SUBCELLULAR LOCALIZATION extracellular
        intracellular
    text
  • extracellular matrix
  • showed extensive co-localization with fibrillin-1 and elastic lamellae (Hirani 2007)
  • basic FUNCTION
  • TGF-beta is released from the extracellular matrix by proteases, localized to cell surfaces
  • may play an integral structural role in elastic-fiber architectural organization and/or assembly
  • targeting latent TGF-beta to the matrix
  • having possibility to compete with LTBP1 for binding to fibrillin-containing microfibrils throughout the aortic wall but particularly in the outer medial region where the LTBP1 is predominantly located (Hirani 2007)
  • may be an indirect negative modulator for storage of the large latent TGF-beta complex on microfibrils in aorta and other fibrillin-rich tissues (Hirani 2007)
  • essential for normal development of the anterior chamber of the eye, where it may have a structural role in maintaining ciliary muscle tone (Ali 2009)
  • may have a negative regulatory role during elastic fiber assembly, perhaps in displacing elastin microassemblies from complexes with FBLN5 and/or cell surface heparan sulfate proteoglycans
  • prominent pericellular component of annular fibrochondrocytes in the posterior annulus fibrosus (AF), interstitial matrix adjacent to nucleus pulposus (NP) cells
  • is an essential component for the formation of microfibril bundles in ciliary zonules
  • LTBP1 and LTBP2 are involved in the keratinization of oral epithelium
  • is a potent inhibitor of FGF2 that may influence FGF2 bioactivity during wound repair particularly in fibrotic tissues
  • plays vital roles in tumorigenesis through regulating TGFB1 activity, elastogenesis and maintenance of the extracellular matrix (ECM) structure
  • acts as an oncogene in head and neck squamous cell carcinoma development and progression
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • not bound to latent TGFB1 independent component of microfibrils
  • component of LAP which is a homodimer formed from the propeptide TGF-beta
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • specifically binds to the amino-terminal region of fibrillin-1, but not to fibrillin-2 (Hirani 2007)
  • LTBP2, LTBP3 inhibit the proprotein convertase PCSK5
  • in response to tension stress, may negatively control the function of FBLN5, thereby modulating the mechanism of oxytalan fiber coalescence
  • LTBP2 is a potent inhibitor of FGF2 that may influence FGF2 bioactivity during wound repair particularly in fibrotic tissues
  • cell & other
  • has widespread association with fibrillin-containing microfibrils in developing aorta and other tissues (Hirani 2007)
  • REGULATION
    activated by cell-associated plasmin
    ASSOCIATED DISORDERS
    corresponding disease(s) GLC3D , MSPKA , WMS3
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional germinal mutation      
    in Marfan syndrome
    tumoral     --over  
    in pancreatic ductal adenocarcinoma (PDAC) tissue
    Susceptibility to BMD variation (bone mineral density) in trochanter and total hip
    Variant & Polymorphism other strong genetic association between LTBP2 gene locus and total hip BMD variation
    Candidate gene
    Marker
  • LTBP2 powerfully predicted early pulmonary death (LTBP2 levels were significantly higher in patients dying from pulmonary causes)
  • serum LTBP2 levels might act as a promising biomarker in heart failure with reduced ejection fraction (HFREF)
  • is a novel biomarker for the diagnosis of Pancreatic cancer
  • Therapy target
    SystemTypeDisorderPubmed
    cancerendocrinepancreas
    may be a potential target for PDAC clinical therapy
    ANIMAL & CELL MODELS