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Mouse Anti-FBLN5 Recombinant Antibody (CBXF-2577) (CBMAB-F3024-CQ)

This product is a mouse antibody that recognizes FBLN5. The antibody CBXF-2577 can be used for immunoassay techniques such as: WB, IHC.
See all FBLN5 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXF-2577
Antibody Isotype
IgG1
Application
WB, IHC

Basic Information

Immunogen
NS0-derived recombinant human Fibulin 5/DANCE, Gln24-Phe448, Accession # Q9UBX5
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Lyophilized
Buffer
Lyophilized from a 0.2 μm filtered solution in PBS with trehalose
Concentration
LYOPH
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Fibulin 5
Introduction
The protein encoded by this gene is a secreted, extracellular matrix protein containing an Arg-Gly-Asp (RGD) motif and calcium-binding EGF-like domains. It promotes adhesion of endothelial cells through interaction of integrins and the RGD motif. It is prominently expressed in developing arteries but less so in adult vessels. However, its expression is reinduced in balloon-injured vessels and atherosclerotic lesions, notably in intimal vascular smooth muscle cells and endothelial cells. Therefore, the protein encoded by this gene may play a role in vascular development and remodeling. Defects in this gene are a cause of autosomal dominant cutis laxa, autosomal recessive cutis laxa type I (CL type I), and age-related macular degeneration type 3 (ARMD3).
Entrez Gene ID
10516
UniProt ID
Q9UBX5
Alternative Names
Fibulin 5; Developmental Arteries And Neural Crest EGF-Like Protein; Urine P50 Protein; FIBL-5; DANCE; UP50; Testis Tissue Sperm-Binding Protein Li 75n;
Research Area
Essential for elastic fiber formation, is involved in the assembly of continuous elastin (ELN) polymer and promotes the interaction of microfibrils and ELN (PubMed:18185537).

Stabilizes and organizes elastic fibers in the skin, lung and vasculature (By similarity).

Promotes adhesion of endothelial cells through interaction of integrins and the RGD motif. Vascular ligand for integrin receptors which may play a role in vascular development and remodeling (PubMed:10428823).

May act as an adapter that mediates the interaction between FBN1 and ELN (PubMed:17255108).
Biological Process
Cell-matrix adhesion Source: ProtInc
Elastic fiber assembly Source: UniProtKB
Protein localization to cell surface Source: BHF-UCL
Regulation of cell growth Source: Ensembl
Regulation of removal of superoxide radicals Source: BHF-UCL
Secretion Source: UniProtKB
Cellular Location
Secreted; Extracellular matrix. Co-localizes with ELN in elastic fibers.
Involvement in disease
Neuropathy, hereditary, with or without age-related macular degeneration (HNARMD):
An autosomal dominant neuropathy of the Charcot-Marie-Tooth disease group, characterized by distal muscle weakness and atrophy variably affecting the lower and upper limbs. Distal sensory impairment and decreased nerve conduction velocities are present in most but not all patients. Additional variable features are age-related macular degeneration, joint hypermobility, and hyperelastic skin.
Cutis laxa, autosomal dominant, 2 (ADCL2):
A connective tissue disorder characterized by loose, hyperextensible skin with decreased resilience and elasticity leading to a premature aged appearance. Face, hands, feet, joints, and torso may be differentially affected. Additional variable clinical features are gastrointestinal diverticula, hernia, and genital prolapse. Rare manifestations are pulmonary artery stenosis, aortic aneurysm, bronchiectasis, and emphysema.
Cutis laxa, autosomal recessive, 1A (ARCL1A):
A connective tissue disorder characterized by loose, hyperextensible skin with decreased resilience and elasticity leading to a premature aged appearance. Face, hands, feet, joints, and torso may be differentially affected. The clinical spectrum of autosomal recessive cutis laxa is highly heterogeneous with respect to organ involvement and severity. Type I autosomal recessive cutis laxa is a specific, life-threatening disorder with organ involvement, lung atelectasis and emphysema, diverticula of the gastrointestinal and genitourinary systems, and vascular anomalies. Associated cranial anomalies, late closure of the fontanel, joint laxity, hip dislocation, and inguinal hernia have been observed but are uncommon.
Macular degeneration, age-related, 3 (ARMD3):
A form of age-related macular degeneration, a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane.
PTM
N-glycosylated.

Tekmenuray-Unal, A., & Durmaz, C. D. (2022). FBLN5-Related Cutis Laxa Syndrome: A Case with a Novel Variant and Review of the Literature. Molecular Syndromology, 1-8.

Pan, Y. Q., Huang, K. S., Chong, T. H., & Li, J. Y. (2022). LINC01089 blocks malignant progression of thyroid cancer by binding miR-27b-3p to enhance the FBLN5 protein level. Discover Oncology, 13(1), 1-12.

Li, Y., Yang, X., & Lu, D. (2022). Knockdown of ubiquitin-conjugating enzyme E2T (UBE2T) suppresses lung adenocarcinoma progression via targeting fibulin-5 (FBLN5). Bioengineered, 13(5), 11867-11880.

Mao, J., Wang, L., Wu, J., Wang, Y., Wen, H., Zhu, X., ... & Yang, H. (2021). miR-370-3p as a Novel Biomarker Promotes Breast Cancer Progression by Targeting FBLN5. Stem cells international, 2021.

Gharesouran, J., Hosseinzadeh, H., Ghafouri-Fard, S., Jabbari Moghadam, Y., Ahmadian Heris, J., Jafari-Rouhi, A. H., ... & Rezazadeh, M. (2021). New insight into clinical heterogeneity and inheritance diversity of FBLN5-related cutis laxa. Orphanet Journal of Rare Diseases, 16(1), 1-13.

Yuan, R., Li, Y., Yang, B., Jin, Z., Xu, J., Shao, Z., ... & Liu, Y. (2021). LOXL1 exerts oncogenesis and stimulates angiogenesis through the LOXL1-FBLN5/αvβ3 integrin/FAK-MAPK axis in ICC. Molecular Therapy-Nucleic Acids, 23, 797-810.

Li, R., Wu, H., Jiang, H., Wang, Q., Dou, Z., Ma, H., ... & Kong, B. (2020). FBLN5 is targeted by microRNA‑27a‑3p and suppresses tumorigenesis and progression in high‑grade serous ovarian carcinoma. Oncology reports, 44(5), 2143-2151.

Padhy, B., Kapuganti, R. S., Hayat, B., Mohanty, P. P., & Alone, D. P. (2019). De novo variants in an extracellular matrix protein coding gene, fibulin-5 (FBLN5) are associated with pseudoexfoliation. European Journal of Human Genetics, 27(12), 1858-1866.

Van Maldergem, L., & Loeys, B. (2018). FBLN5-related cutis laxa. GeneReviews®[Internet].

Davila, N., Hernandez, L. F., Corredor, C., Perez-Vazquez, J. F. F., Soberón, S., Enriquez, A., ... & Quiroz-Mercado, H. (2018). Expression levels of Fibulin-2 (Fbln2) and Fibulin-5 (Fbln5) on subretinal fluid in human primary rhegmatogenous retinal detachment. Investigative Ophthalmology & Visual Science, 59(9), 4225-4225.

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For research use only. Not intended for any clinical use.

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