LTBP-4 antibody | MyBioSource MBS9402509 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

LTBP-4 antibody

catalog :

MBS9402509

quantity :

0.1 mL

price :

320 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human

application :

western blot, immunocytochemistry

more info or order :

MyBioSource product webpage

image

image 1 :

Sample (30 ug of whole cell lysate) A: Hela B: JurKat 5% SDS PAGE Primary antibody diluted at 1: 1000

image 2 :

Immunofluorescence analysis of methanol-fixed A431, using LTBP4 antibody at 1: 200 dilution.

product information

catalog number :

MBS9402509

products type :

Antibody

products full name :

LTBP-4 antibody

products short name :

[LTBP-4]

other names :

[latent-transforming growth factor beta-binding protein 4 isoform b; Latent-transforming growth factor beta-binding protein 4; latent-transforming growth factor beta-binding protein 4; latent transforming growth factor beta binding protein 4]

products gene name :

[LTBP-4]

other gene names :

[LTBP4; LTBP4; ARCL1C; LTBP-4; LTBP4L; LTBP4S; LTBP-4]

uniprot entry name :

LTBP4_HUMAN

clonality :

Polyclonal

host :

Rabbit

reactivity :

Human

sequence length :

1587

purity :

Purified by antigen-affinity chromatography.

form :

Supplied in 1XPBS, 1%BSA, 20% Glycerol (pH7.0). 0.01% Thimerosal was added as a preservative.

storage stability :

Store at -20 degree C for long term preservation (recommended). Store at 4 degree C for short term use.

tested application :

Western Blot (WB), Immunofluorescence (IF)

app notes :

Western blotting: 1:500-1:3000. Immunofluorescence: 1:100-1:200

image1 heading :

Testing Data

image2 heading :

Immunofluorescence (IF)

other info1 :

Immunogen Type: Recombinant protein. Immunogen Description: Recombinant protein fragment contain a sequence corresponding to a region within amino acids 64 and 256 of LTBP-4

other info2 :

Target Name: LTBP-4

products categories :

Total protein Ab

products description :

Transforming growth factor-beta, or TGFB (see TGFB1; MIM 190180), remains biologically latent after secretion, blocked from activity by its N-terminal propeptide (the latency-associated peptide, or LAP). TGFB is activated by its dissociation from LAP. The latent form of TGFB is secreted in association with a latent TGFB-binding protein, or LTBP (see LTBP1; MIM 150390), and assembled into the extracellular matrix. LTBPs are mainly composed of epidermal growth factor (EGF; MIM 131530)-like repeats and protein domains with 8-cysteine repeats, both of which are also found in fibrillins (e.g., MIM 134797). See Oklu and Hesketh (2000) [PubMed 11104663] for a review of the LTBP gene family.[supplied by OMIM]

ncbi gi num :

110347412

ncbi acc num :

NP_003564

ncbi gb acc num :

NM_003573.2

ncbi mol weight :

Predicted: 169kd

ncbi pathways :

Elastic Fibre Formation Pathway (1270251); Extracellular Matrix Organization Pathway (1270244); Molecules Associated With Elastic Fibres Pathway (1270252)

ncbi summary :

The protein encoded by this gene binds transforming growth factor beta (TGFB) as it is secreted and targeted to the extracellular matrix. TGFB is biologically latent after secretion and insertion into the extracellular matrix, and sheds TGFB and other proteins upon activation. Defects in this gene may be a cause of cutis laxa and severe pulmonary, gastrointestinal, and urinary abnormalities. Three transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, May 2010]

uniprot summary :

LTBP4: 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). Defects in LTBP4 are the cause of Urban-Rifkin-Davis syndrome (URDS); 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. Belongs to the LTBP family. 4 isoforms of the human protein are produced by alternative splicing. Protein type: Extracellular matrix; Secreted, signal peptide; Secreted. Chromosomal Location of Human Ortholog: 19q13.1-q13.2. Cellular Component: extracellular matrix; extracellular space; proteinaceous extracellular matrix; extracellular region. Molecular Function: integrin binding; transforming growth factor beta receptor activity; protein binding; glycosaminoglycan binding; transforming growth factor beta binding; calcium ion binding. Biological Process: growth hormone secretion; extracellular matrix organization and biogenesis; regulation of transforming growth factor beta receptor signaling pathway; protein folding; regulation of cell differentiation; multicellular organismal development; transforming growth factor beta receptor signaling pathway; regulation of proteolysis; regulation of cell growth. Disease: Cutis Laxa, Autosomal Recessive, Type Ic

size1 :

0.1 mL

price1 :

320 USD

more info or order :

MyBioSource product webpage