LAMA2 Antibody
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中文名稱：LAMA2兔多克隆抗體
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貨號：CSB-PA009763
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規(guī)格：￥1090
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其他：
產品詳情
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Uniprot No.：
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基因名：
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別名：LAMA 2 antibody; LAMA2 antibody; LAMA2_HUMAN antibody; Laminin alpha 2 (merosin congenital muscular dystrophy) antibody; Laminin alpha 2 antibody; Laminin alpha 2 chain antibody; Laminin alpha 2 subunit antibody; Laminin M antibody; Laminin M chain antibody; Laminin subunit alpha-2 antibody; Laminin-12 subunit alpha antibody; Laminin-2 subunit alpha antibody; Laminin-4 subunit alpha antibody; LAMM antibody; Merosin heavy chain antibody
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宿主：Rabbit
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反應種屬：Human,Mouse
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免疫原：Synthesized peptide derived from the Internal region of Human Laminin α-2.
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免疫原種屬：Homo sapiens (Human)
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標記方式：Non-conjugated
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抗體亞型：IgG
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純化方式：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
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產品提供形式：Liquid
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應用范圍：IHC, IF, ELISA
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推薦稀釋比：
Application Recommended Dilution IHC 1:100-1:300 IF 1:200-1:1000 ELISA 1:20000 -
Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：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.
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基因功能參考文獻：
- The LAMA2 Mutation Congenital Muscular Dystrophy showed demyelinating polyneuropathy white matter changes on brain. PMID: 29465610
- It was established that the frequency of individuals with the COL13A1*D/*D genotype was higher in the senile age period. The LAMA2*I/*D genotype was predisposing to longevity among women. PMID: 29369589
- Using high-throughput technology identify LAMA-2 as a candidate medullary sponge kidney disease biomarker possibly employable in future for the early diagnosis of this disease. PMID: 27914711
- Differential protein expression of collagen IV, laminin alpha2, and nidogen-1 indicated basal lamina remodeling develops in ischemic failing versus nonfailing human hearts. PMID: 26756417
- Next generation sequencing was found to be useful for molecular diagnosis of a confirmed case of merosin deficient congenital muscular dystrophy. PMID: 26104111
- did not find positive association signals of the four single nucleotide polymorphisms in the LAMA2 and EGR1 genes with high myopia PMID: 26984843
- By analyzing the gene test we found that compound heterozygous LAMA2 mutation inherited from the parents. One coming from the father was a gross deletion expanding from exon 36 to exon 65. The from the mother was a missense mutation c.1358G>C PMID: 26304763
- This report widens the clinical spectrum of cerebral manifestations related with mutations in LAMA2 PMID: 25500573
- Data showed miR-29a/c as novel regulators of LAMA2 in ependymoma based on miRNA-mRNA covariation and sequence-based target predictions. PMID: 25958202
- This study demonstrates a wide clinical spectrum of LAMA2-related muscular dystrophy and its prevalence in an LGMD2 cohort, which indicates that LAMA2 muscular dystrophy should be included in the LGMD2 nomenclature. PMID: 25663498
- Crystal structure of LAMM L4 domain PMID: 25962468
- Data find high frequency mutations in LAMA2 protein in hepatocellular carcinoma (HCC) patients. Its lower expression levels correlate with tumor progression, poor survival and higher chance of cancer recurrence. PMID: 25159915
- Extracellular matrix proteins expression profiling in chemoresistant variants of the A2780 ovarian cancer cell line. PMID: 24804215
- 2 patients with partial laminin-alpha2 deficiency and atypical phenotypes, one with almost exclusive central nervous system involvement and second with cardiac dysfunction, rigid spine syndrome and limb-girdle weakness; both have 2 heterozygous LAMA2 variants sharing a potentially pathogenic missense mutation c.2461A>C located in exon 18 PMID: 24534542
- Genetic association studies identified two pathogenic mutations in the LAMA2 gene in patients with congenital muscular dystrophy. PMID: 24225367
- Homozygous truncating mutations in POMK lead to congenital muscular dystrophies with secondary merosin deficiency, hypomyelination and intellectual disability. PMID: 24556084
- children with LAMA2 congenital muscular dystrophy may be at nogreater risk of developing malignant hyperthermia than the general population PMID: 24628934
- Identification of cell adhesive sequences in the N-terminal region of the laminin alpha2 chain. PMID: 22654118
- Aberrant methylation at target CpG sites in GABRA1 and LAMA2 was observed with high frequency in tumor tissues. PMID: 22038115
- This largest series of patients with limb-girdle muscular dystrophy due to laminin alpha2-deficiency expands the clinical phenotype associated with LAMA2 mutations. PMID: 21953594
- This is the first report to describe dilated cardiomyopathy with conduction defects and merosin deficiency in a patient carrying LAMA2 gene mutations. PMID: 22006699
- A single base deletion at position 8005 in the LAMA2 gene is associated with a severe form of classical congenital muscular dystrophy and partial merosin deficiency in congenital muscular dystrophy type 1A. PMID: 20477750
- LAMA2 mutations were found in three different Russian families with congenital muscular dystrophy. PMID: 20607928
- This large study identified novel LAMA2 mutations and highlights the role of immunohistochemical studies for merosin status in predicting clinical severity of MDC1A. PMID: 20207543
- Data show that the expression of collagen types I, III and fibronectin was significantly higher in pancreatic cancer, and the expression of collagen type IV, laminin and vitronectin was significantly lower in pancreatic cancer. PMID: 19893454
- Ku70 is a regulator of Bax-mediated pathogenesis in laminin-alpha2-deficiency muscle cells. PMID: 19692349
- case series and review of laminin alpha2(merosin) deficiency phenotypes and abnormalities PMID: 11584042
- A number of mutations are identified in association with congenital muscular dystrophies. PMID: 11938437
- Merosin-deficient congenital muscular dystrophy with mental retardation and cerebellar cysts, unlinked to the LAMA2 locus in three Tunisian patients. PMID: 12467726
- In nine congenital muscular dystrophy patients with abnormal white-matter signal at brain MRI and partial deficiency of muscle laminin alpha 2, three novel missense and two splice-site mutations were found. PMID: 12552556
- However, dy(W)/dy(W) mice, expressing the human laminin alpha2 under the control of the striated muscle-specific portion of the desmin promoter, still developed muscular dystrophy. PMID: 12609502
- A mild case of autosomal recessive congenital muscular dystrophy is associated with a homozygous out-of-frame deletion in exon 56 of the LAMA2 gene. PMID: 15452315
- DNA analysis can be used to provide accurate prenatal diagnosis of thecongenital muscular dystrophy, and have an essential role in genetic counseling. PMID: 16084089
- identified 9 new LAMA 2 mutations PMID: 16216942
- A relocalization of LAMA2 was noted in the subepithelial basement membrane in a group of Hirschsprung patients. PMID: 16226104
- suggest that LNalpha1 chain in part ameliorates the development of LNalpha2 chain deficient muscular dystrophy by retaining the binding sites for integrin alpha7Bbeta1D and alpha-dystroglycan, respectively PMID: 16504180
- Study summarizes recent progress concerning the molecular mechanisms of laminins in development and disease. PMID: 17426950
- intron mutation is responsible for complete exon 17 skipping in severe congenital muscular dystrophy PMID: 18053718
- the first fully characterized gross deletion in the LAMA2 gene, encompassing exon 56 (c.7750-1713_7899-2153del), detected in 31% of congenital muscular dystrophy type 1A patients PMID: 18700894
- In patient 1, a double mutation, c.[9101_9104dupAACA:3412G>A] p.[H3035QfsX4:V1138M] was detected, Patient 2 had a novel homozygous nonsense mutation, c.2907C>A (p.Cys969X), in exon 21. PMID: 19294599
- Crystal structure shows that the three LG domains adopt typical beta-sandwich folds, with canonical calcium binding sites in LG1 and LG2. LG2 and LG3 interact through a substantial interface, but LG1 is completely dissociated from the LG2-3 pair. PMID: 19553699
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相關疾病：Merosin-deficient congenital muscular dystrophy 1A (MDC1A)
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亞細胞定位：Secreted, extracellular space, extracellular matrix, basement membrane. Note=Major component.
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組織特異性：Placenta, striated muscle, peripheral nerve, cardiac muscle, pancreas, lung, spleen, kidney, adrenal gland, skin, testis, meninges, choroid plexus, and some other regions of the brain; not in liver, thymus and bone.
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