LTBP1

Latent transforming growth factor beta binding protein 1 functions in aorta development, coronary vasculature development, positive regulation of collagen biosynthetic process, transforming growth factor beta receptor signaling pathway and ventricular septum development.
Full Name
latent transforming growth factor beta binding protein 1
Function
Key regulator of transforming growth factor beta (TGFB1, TGFB2 and TGFB3) that controls TGF-beta activation by maintaining it in a latent state during storage in extracellular space (PubMed:2022183, PubMed:8617200, PubMed:8939931).
Associates specifically via disulfide bonds with the Latency-associated peptide (LAP), which is the regulatory chain of TGF-beta, and regulates integrin-dependent activation of TGF-beta (PubMed:8617200, PubMed:8939931, PubMed:15184403).
Outcompeted by LRRC32/GARP for binding to LAP regulatory chain of TGF-beta (PubMed:22278742).
Biological Process
Regulation of transforming growth factor beta activationManual Assertion Based On ExperimentIDA:UniProtKB
Sequestering of TGFbeta in extracellular matrixManual Assertion Based On ExperimentIDA:UniProtKB
Cellular Location
Secreted
Secreted, extracellular space, extracellular matrix
Involvement in disease
Cutis laxa, autosomal recessive, 2E (ARCL2E):
A form of cutis laxa, a disorder characterized by an excessive congenital skin wrinkling, a large fontanelle with delayed closure, a typical facial appearance with downslanting palpebral fissures, and a general connective tissue weakness. ARCL2E patients present with cutis laxa, inguinal hernia, craniofacial dysmorphology, variable heart defects, and prominent skeletal features including craniosynostosis, short stature, brachydactyly, and syndactyly.
PTM
Contains hydroxylated asparagine residues.
Isoform Short N-terminus is blocked.
Two intrachain disulfide bonds from the TB3 domain are rearranged upon TGFB1 binding, and form interchain bonds with TGFB1 propeptide, anchoring it to the extracellular matrix.
O-glycosylated on serine residues by POGLUT2 and POGLUT3.
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Anti-LTBP1 antibodies

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Mouse Anti-LTBP1 Recombinant Antibody (MM0447-5L38) (CBMAB-L2326-YJ)

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Target: LTBP1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: MM0447-5L38
Application*: WB
Datasheet SDS

Mouse Anti-LTBP1 Recombinant Antibody (5D7) (CBMAB-L2324-YJ)

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Target: LTBP1
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human
Clone: 5D7
Application*: E, WB
Datasheet SDS

Mouse Anti-LTBP1 Recombinant Antibody (CBYJL-2149) (CBMAB-L2323-YJ)

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Target: LTBP1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBYJL-2149
Application*: IS
Datasheet SDS

Mouse Anti-LTBP1 Recombinant Antibody (CBYJL-2147) (CBMAB-L2321-YJ)

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Target: LTBP1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBYJL-2147
Application*: WB
Datasheet SDS

Human LTBP1 ELISA Kit (2) (V2LY-0626-LY5927)

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Target: LTBP1
Sensitivity: 0.0054 ng/mL
Detection Range: 0.01-3 ng/mL
Sample Type: Serum, Plasma, cell culture supernates
Specificity: Human
Assay Type: Sandwich
Reactivity: Human
Datasheet SDS

Human LTBP1 ELISA Kit (V2LY-0626-LY1976)

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Target: LTBP1
Sensitivity: 0.023 ng/mL
Detection Range: 0.05-20 ng/mL
Sample Type: Serum, Plasma, cell culture supernates
Specificity: Human
Assay Type: Sandwich
Reactivity: Human
Datasheet SDS

Rat Ltbp1 ELISA Kit (V2LY-0626-LY1285)

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Target: Ltbp1
Sensitivity: 0.0091 ng/mL
Detection Range: 0.02-4.5 ng/mL
Sample Type: Serum, Plasma, cell culture supernates
Specificity: Rat
Assay Type: Sandwich
Reactivity: Rat
Datasheet SDS
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(P): Predicted
* Abbreviations
  • AActivation
  • AGAgonist
  • APApoptosis
  • BBlocking
  • BABioassay
  • BIBioimaging
  • CImmunohistochemistry-Frozen Sections
  • CIChromatin Immunoprecipitation
  • CTCytotoxicity
  • CSCostimulation
  • DDepletion
  • DBDot Blot
  • EELISA
  • ECELISA(Cap)
  • EDELISA(Det)
  • ESELISpot
  • EMElectron Microscopy
  • FFlow Cytometry
  • FNFunction Assay
  • GSGel Supershift
  • IInhibition
  • IAEnzyme Immunoassay
  • ICImmunocytochemistry
  • IDImmunodiffusion
  • IEImmunoelectrophoresis
  • IFImmunofluorescence
  • IGImmunochromatography
  • IHImmunohistochemistry
  • IMImmunomicroscopy
  • IOImmunoassay
  • IPImmunoprecipitation
  • ISIntracellular Staining for Flow Cytometry
  • LALuminex Assay
  • LFLateral Flow Immunoassay
  • MMicroarray
  • MCMass Cytometry/CyTOF
  • MDMeDIP
  • MSElectrophoretic Mobility Shift Assay
  • NNeutralization
  • PImmunohistologyp-Paraffin Sections
  • PAPeptide Array
  • PEPeptide ELISA
  • PLProximity Ligation Assay
  • RRadioimmunoassay
  • SStimulation
  • SESandwich ELISA
  • SHIn situ hybridization
  • TCTissue Culture
  • WBWestern Blot
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