latent transforming growth factor beta binding protein 4; latent transforming growth factor beta binding protein 4L; Latent-transforming growth factor beta-binding protein 4; LTBP 4; LTBP-4; LTBP4; LTBP4_HUMAN; LTBP4L; LTBP4S.
Cat:
SL5779R
Species Reactivity：
(predicted: Human,Mouse,Rat,Dog,Pig,Cow,Sheep,)
Immunogen：
KLH conjugated synthetic peptide derived from human LTBP4:151-250/1624
Format：
Liquid
Storage instructions：
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Concentration：
1mg/ml
Clonality：
Polyclonal
Isotype：
IgG
Applications：
WB=1:500-2000ELISA=1:5000-10000IHC-P=1:100-500IHC-F=1:100-500IF=1:100-500（Paraffin sections need to do antigen repair）not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
May be involved in the assembly, secretion and targeting of TGFB1 to sites at which it is stored and/or activated. May play critical roles in controlling and directing the activity of TGFB1. May have a structural role in the extra cellular matrix (ECM).
Function: May be involved in the assembly, secretion and targeting of TGFB1 to sites at which it is stored and/or activated. May play critical roles in controlling and directing the activity of TGFB1. May have a structural role in the extra cellular matrix (ECM) (By similarity).
Subunit: Forms part of the large latent transforming growth factor beta precursor complex; removal is essential for activation of complex. Interacts with LTBP1 and TGFB1. Binds to FBN1 (By similarity).
DISEASE: Defects in LTBP4 are the cause of Urban-Rifkin-Davis syndrome (URDS) [MIM:613177]; also known as Cutis laxa with severe pulmonary gastrointestinal and urinary abnormalities. URDS is a syndrome characterized by disrupted pulmonary, gastrointestinal, urinary, musculoskeletal, craniofacial and dermal development. Clinical features include cutis laxa, mild cardiovascular lesions, respiratory distress with cystic and atelectatic changes in the lungs, and diverticulosis, tortuosity and stenosis at various levels of the intestinal tract. Craniofacial features include microretrognathia, flat midface, receding forehead and wide fontanelles.
Similarity: Belongs to the LTBP family. Contains 16 EGF-like domains. Contains 4 TB (TGF-beta binding) domains.
Specific References (1) | SL5779R has been referenced in 1 publications.
[IF=11.47] Choi, Won Hoon, et al. "Open-gate mutants of the mammalian proteasome show enhanced ubiquitin-conjugate degradation." Nature Communications 7 (2016). WB ; Human.
