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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
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