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Rat Anti-Lamb2 Recombinant Antibody (A5) (CBMAB-L2713-YJ)


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Summary

Host Animal
Rat
Specificity
Mouse
Clone
A5
Antibody Isotype
IgG2a, κ
Application
IF, IHC-Fr, IP

Basic Information

Specificity
Mouse
Antibody Isotype
IgG2a, 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
10 mM PBS, pH 7.4, 0.2% BSA
Preservative
0.09% Sodium Azide
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
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. Lamb2 is laminin subunit beta-2.
Entrez Gene ID
16779
UniProt ID
Q61292
Alternative Names
Lams; npht; Lamb-2; AW211941; laminin subunit beta-2; Laminin S; S-LAM beta; S-laminin subunit beta; laminin-11 subunit beta; laminin-14 subunit beta; laminin-15 subunit beta; laminin-3 subunit beta; laminin-4 subunit beta; laminin-7 subunit beta; laminin-9 subunit beta
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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