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Rat Anti-LAMB2 Recombinant Antibody (CBYJL-1110) (CBMAB-L0575-YJ)


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Summary

Host Animal
Rat
Specificity
Human, Mouse
Clone
CBYJL-1110
Antibody Isotype
IgG2, κ
Application
IHC

Basic Information

Immunogen
Murine EHS laminin.
Specificity
Human, Mouse
Antibody Isotype
IgG2, κ
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!]

Buffer
0.1 M Tris-glycine, pH 7.0
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
LAMB2
Introduction
Laminins, a family of extracellular matrix glycoproteins, are the major noncollagenous constituent of basement membranes. They have been implicated in a wide variety of biological processes including cell adhesion, differentiation, migration, signaling, neurite outgrowth and metastasis. Laminins, composed of 3 non identical chains: laminin alpha, beta and gamma (formerly A, B1, and B2, respectively), form a cruciform structure consisting of 3 short arms, each formed by a different chain, and a long arm composed of all 3 chains. Each laminin chain is a multidomain protein encoded by a distinct gene. Several isoforms of each chain have been described. Different alpha, beta and gamma chain isomers combine to give rise to different heterotrimeric laminin isoforms which are designated by Arabic numerals in the order of their discovery, i.e. alpha1beta1gamma1 heterotrimer is laminin 1. The biological functions of the different chains and trimer molecules are largely unknown, but some of the chains have been shown to differ with respect to their tissue distribution, presumably reflecting diverse functions in vivo. LAMB2 is the beta chain isoform laminin, beta 2. The beta 2 chain contains the 7 structural domains typical of beta chains of laminin, including the short alpha region. However, unlike beta 1 chain, beta 2 has a more restricted tissue distribution. It is enriched in the basement membrane of muscles at the neuromuscular junctions, kidney glomerulus and vascular smooth muscle. Transgenic mice in which the beta 2 chain gene was inactivated by homologous recombination, showed defects in the maturation of neuromuscular junctions and impairment of glomerular filtration. Alternative splicing involving a non consensus 5' splice site (gc) in the 5' UTR of this gene has been reported. It was suggested that inefficient splicing of this first intron, which does not change the protein sequence, results in a greater abundance of the unspliced form of the transcript than the spliced form. The full-length nature of the spliced transcript is not known. Among its related pathways are ERK Signaling and Focal Adhesion.
Entrez Gene ID
Human3913
Mouse16779
UniProt ID
HumanP55268
MouseQ61292
Alternative Names
LAMS; NPHS5
Function
Binding to cells via a high affinity receptor, laminin is thought to mediate the attachment, migration and organization of cells into tissues during embryonic development by interacting with other extracellular matrix components.
Biological Process
Animal organ morphogenesisManual Assertion Based On ExperimentIBA:GO_Central
Astrocyte developmentIEA:Ensembl
Axon extension involved in regenerationIEA:Ensembl
Axon guidanceIEA:Ensembl
Basement membrane assemblyManual Assertion Based On ExperimentIBA:GO_Central
Cell migrationManual Assertion Based On ExperimentIBA:GO_Central
Metanephric glomerular basement membrane developmentIEA:Ensembl
Metanephric glomerular visceral epithelial cell developmentIEA:Ensembl
Neuromuscular junction developmentIEA:Ensembl
Retina development in camera-type eyeIEA:Ensembl
Schwann cell developmentIEA:Ensembl
Substrate adhesion-dependent cell spreadingManual Assertion Based On ExperimentIBA:GO_Central
Tissue developmentManual Assertion Based On ExperimentIBA:GO_Central
Visual perceptionIEA:Ensembl
Cellular Location
Secreted, extracellular space, extracellular matrix, basement membrane
S-laminin is concentrated in the synaptic cleft of the neuromuscular junction.
Involvement in disease
Pierson syndrome (PIERSS):
Characterized by nephrotic syndrome with neonatal onset, diffuse mesangial sclerosis and eye abnormalities with microcoria as the leading clinical feature. Death usually occurs within the first weeks of life. Disease severity depends on the mutation type: nontruncating LAMB2 mutations may display variable phenotypes ranging from a milder variant of Pierson syndrome to isolated congenital nephrotic syndrome.
Nephrotic syndrome 5 with or without ocular abnormalities (NPHS5):
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure. NPHS5 is characterized by very early onset of progressive renal failure. A subset of patients may develop mild ocular anomalies, such as myopia, nystagmus, and strabismus.
More Infomation

Suzuki, R., Sakakibara, N., Ichikawa, Y., Kitakado, H., Ueda, C., Tanaka, Y., ... & Nozu, K. (2023). Systematic Review of Clinical Characteristics and Genotype-Phenotype Correlation in LAMB2-Associated Disease. Kidney International Reports.

Alshamrani, A. A., Magliyah, M., Alkuraya, F. S., Alabdi, L., Alfaadhel, T. A., & Alsulaiman, S. M. (2023). Early-Onset Myopia and Retinal Detachment without Typical Microcoria or Severe Proteinuria due to a Novel LAMB2 Variant. Ophthalmology Retina.

Cheng, N., Xiong, Y., Zhang, W., Wu, X., Sun, Z., Zhang, L., ... & Peng, Y. (2022). Astrocytes promote the proliferation of oligodendrocyte precursor cells through connexin 47-mediated LAMB2 secretion in exosomes. Molecular Biology Reports, 49(8), 7263-7273.

Thakor, J. M., Parmar, G., Mistry, K. N., Gang, S., Rank, D. N., & Joshi, C. G. (2021). Mutational landscape of TRPC6, WT1, LMX1B, APOL1, PTPRO, PMM2, LAMB2 and WT1 genes associated with Steroid resistant nephrotic syndrome. Molecular Biology Reports, 48, 7193-7201.

Wang, X., Xiao, H., Su, B., Ren, Y., Ding, J., & Wang, F. (2021). LAMB2 novel variant c. 2885‐9 C> A affects RNA splicing in a minigene assay. Molecular Genetics & Genomic Medicine, 9(7), e1704.

Tahoun, M., Chandler, J. C., Ashton, E., Haston, S., Hannan, A., Kim, J. S., ... & Waters, A. M. (2020). Mutations in LAMB2 are associated with albuminuria and optic nerve hypoplasia with hypopituitarism. The Journal of Clinical Endocrinology & Metabolism, 105(3), 595-599.

Jing, F., Zhao, J., Jing, X., & Lei, G. (2020). Long noncoding RNA Airn protects podocytes from diabetic nephropathy lesions via binding to Igf2bp2 and facilitating translation of Igf2 and Lamb2. Cell Biology International, 44(9), 1860-1869.

Bachay, G., Umino, Y., Solessio, E. C., Noguera, F., Watters, J., Hunter, D. D., & Brunken, W. J. (2020). Physiological And Anatomical Role Of Neural Derived Lamb2 In The Retina. Investigative Ophthalmology & Visual Science, 61(7), 685-685.

Zhu, H. T., Maimaiti, M., Cao, C., Luo, Y. F., Julaiti, D., Liang, L., & Abudureheman, A. (2019). A novel homozygous truncating mutation in LAMB2 gene in a Chinese Uyghur patient with severe phenotype Pierson syndrome. Frontiers in Medicine, 6, 12.

Abid, A., Shahid, S., Shakoor, M., Lanewala, A. A., Hashmi, S., & Khaliq, S. (2018). Screening of the LAMB2, WT1, NPHS1, and NPHS2 genes in pediatric nephrotic syndrome. Frontiers in Genetics, 9, 214.

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

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