MYOT Antibody
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中文名稱:MYOT兔多克隆抗體
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貨號:CSB-PA015364DSR2HU
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規(guī)格:¥440
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圖片:
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其他:
產(chǎn)品詳情
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產(chǎn)品名稱:Rabbit anti-Homo sapiens (Human) MYOT Polyclonal antibody
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Uniprot No.:
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基因名:MYOT
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別名:57 kDa cytoskeletal protein antibody; LGMD 1 antibody; LGMD1 antibody; Myofibrillar titin like Ig domains protein antibody; Myofibrillar titin-like Ig domains protein antibody; Myot antibody; MYOTI_HUMAN antibody; Myotilin antibody; Titin immunoglobulin domain protein antibody; TTID antibody; TTID protein antibody
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宿主:Rabbit
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反應(yīng)種屬:Human
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免疫原:Recombinant Human Myotilin protein (443-498AA)
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免疫原種屬:Homo sapiens (Human)
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標(biāo)記方式:Non-conjugated
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克隆類型:Polyclonal
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抗體亞型:IgG
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純化方式:Antigen Affinity Purified
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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:PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
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產(chǎn)品提供形式:Liquid
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應(yīng)用范圍:ELISA, IHC
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推薦稀釋比:
Application Recommended Dilution IHC 1:20-1:200 -
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.
相關(guān)產(chǎn)品
靶點詳情
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功能:Component of a complex of multiple actin cross-linking proteins. Involved in the control of myofibril assembly and stability at the Z lines in muscle cells.
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基因功能參考文獻:
- sequence conservation analysis of myotilin shed light on the molecular basis of myotilinopathies and revealed several motifs in Ig domains found also in I-band proteins. PMID: 28638118
- A French family affected with a late onset proximal and distal muscle weakness and myofibrillar myopathy on muscle pathology, in which the siblings known to be clinically affected were homozygous for the c.179C>T (p.Ser60Phe) myotilin gene mutation is reported. PMID: 27854214
- Describe the first homozygous mutation in the myotilin gene leading to a novel, autosomal recessive subtype of myofibrillar myopathy (MFM). PMID: 24928145
- Analysis of myotilin turnover provides mechanistic insight into the role of myotilinopathy-causing mutations PMID: 21361873
- A second known pedigree with LGMD1A: this finding constitutes a gold standard of proof that mutations in the myotilin gene cause Limb-Girdle Muscular Dystrophy 1A PMID: 12428213
- Myotilin a thin filament-associated Z-disc protein.It binds to alpha-actinin and filamin c and is mutated in limb girdle muscular dystrophy 1A (LGMD1A).myotilin binds F-actin and prevents filament disassembly induced by Latrunculin A PMID: 12499399
- Mutations in myotilin cause MFM; exon 2 of MYOT is a hotspot for mutations; peripheral neuropathy, cardiomyopathy, and distal weakness greater than proximal weakness are part of the spectrum of myotilinopathy; not all cases have a limb-girdle phenotype PMID: 15111675
- Our findings provide evidence for a novel connection between the Z-disc protein myotilin and the sarcolemma via filamins and beta1 integrins. PMID: 16076904
- The function of the myotilin protein is studied with regards its actin-organizing properties. PMID: 16122733
- A novel mutation in the myotilin gene results in the clinical and pathologic phenotype termed "spheroid body myopathy." Mutations in this gene also cause limb-girdle muscular dystrophy 1A and are associated with myofibrillar myopathy. PMID: 16380616
- Mutations within the MYOT gene are not a cause for Vocal Cord and Pharyngeal Weakness with Distal Myopathy (VCPDM). PMID: 16674563
- multigenerational French family in which gene sequencing identified a S60F myotilin mutation in all patients with full penetrance despite very late onset PMID: 16793270
- Myotilin mutations promote aggregate-dependent contractile dysfunction similar to Limb-girdle Muscular Dystrophy type 1A and Myofibrillar Myopathy. PMID: 16801328
- Myotilin S55F mutations may cause a clinically distinct autosomal-dominant late-onset and lower-limb distal myopathic syndrome. MRI helps to depict the topography of fatty muscle atrophy and to detect gene mutation carriers. PMID: 17698502
- new autosomal dominant kindred with generalized symmetrical increase in muscle bulk PMID: 19027924
- This is the first report of a binding motif common to both the myotilin and the FATZ (calsarcin/myozenin) families that is specific for interactions with Enigma family members. PMID: 19047374
- Data show that in myofibrillar myopathies myotilin exhibites significant alterations in their localization. PMID: 19151983
- study presents high-resolution structure of the first Ig-domain of myotilin determined with solution state NMR spectroscopy; structure of MyoIg1 exhibits I-type Ig-fold PMID: 19418025
- identified a novel MYOT mutation in Exon 9 encoding the second immunoglobulin-like domain in 1 patient with clinically typical limb girdle muscular dystrophy PMID: 19458539
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相關(guān)疾病:Limb-girdle muscular dystrophy 1A (LGMD1A); Myopathy, myofibrillar, 3 (MFM3); Spheroid body myopathy (SBM)
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亞細胞定位:Cell membrane, sarcolemma. Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere, Z line.
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蛋白家族:Myotilin/palladin family
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組織特異性:Expressed in skeletal muscle (at protein level). Expressed in skeletal muscle, heart, bone marrow and thyroid gland.
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