Anti-LAMB2 Antibody (CAB10565)
- SKU:
- CAB10565
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
| 抗体名: | Anti-LAMB2 Antibody |
| 抗体コード: | CAB10565 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB IF |
| 反応性: | Human, Mouse |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1579-1798 of human LAMB2 (NP_002283.3). |
| 申し込み: | WB IF |
| 推奨希釈: | WB 1:1000 - 1:2000 IF 1:50 - 1:200 |
| 反応性: | Human, Mouse |
| ポジティブサンプル: | LO2 |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1579-1798 of human LAMB2 (NP_002283.3). |
| 精製方法: | Affinity purification |
| ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| アイソタイプ: | IgG |
| 順序: | DVRR AEQL LQDA RRAR SWAE DEKQ KAET VQAA LEEA QRAQ GIAQ GAIR GAVA DTRD TEQT LYQV QERM AGAE RALS SAGE RARQ LDAL LEAL KLKR AGNS LAAS TAEE TAGS AQGR AQEA EQLL RGPL GDQY QTVK ALAE RKAQ GVLA AQAR AEQL RDEA RDLL QAAQ DKLQ RLQE LEGT YEEN ERAL ESKA AQLD GLEA RMRS VLQA INLQ VQIY NTCQ |
| 遺伝子ID: | 3913 |
| Uniprot: | P55268 |
| セルラーロケーション: | Secreted, basement membrane, extracellular matrix, extracellular space |
| 計算された分子量: | 195kDa |
| 観察された分子量: | 196kDa |
| 同義語: | LAMB2, LAMS, NPHS5 |
| バックグラウンド: | 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. This gene encodes 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. |
| UniProt Protein Function: | LAMB2: 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. Defects in LAMB2 are the cause of Pierson syndrome (PIERSS); also known as microcoria-congenital nephrotic syndrome. Pierson syndrome is 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. Defects in LAMB2 are the cause of nephrotic syndrome type 5 with or without ocular abnormalities (NPHS5). NPHS5 is a renal disease characterized clinically by proteinuria, 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. |
| UniProt Protein Details: | Protein type:Extracellular matrix; Motility/polarity/chemotaxis; Secreted; Secreted, signal peptide Chromosomal Location of Human Ortholog: 3p21.31 Cellular Component: basal lamina; basement membrane; extracellular matrix; extracellular region; laminin-3 complex Biological Process: extracellular matrix organization and biogenesis Disease: Nephrotic Syndrome, Type 5, With Or Without Ocular Abnormalities; Pierson Syndrome |
| NCBI Summary: | 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. This gene encodes 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. [provided by RefSeq, Aug 2011] |
| UniProt Code: | P55268 |
| NCBI GenInfo Identifier: | 156630892 |
| NCBI Gene ID: | 3913 |
| NCBI Accession: | P55268.2 |
| UniProt Secondary Accession: | P55268,Q16321, |
| UniProt Related Accession: | P55268 |
| Molecular Weight: | 196kDa |
| NCBI Full Name: | Laminin subunit beta-2 |
| NCBI Synonym Full Names: | laminin subunit beta 2 |
| NCBI Official Symbol: | LAMB2 |
| NCBI Official Synonym Symbols: | LAMS; NPHS5 |
| NCBI Protein Information: | laminin subunit beta-2 |
| UniProt Protein Name: | Laminin subunit beta-2 |
| UniProt Synonym Protein Names: | Laminin B1s chain; 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; S-laminin subunit beta; S-LAM beta |
| Protein Family: | Laminin |
| UniProt Gene Name: | LAMB2 |
 | Western blot analysis of extracts of LO2 cells, using LAMB2 antibody (CAB10565) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 5s. |
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