Anti-Myotilin抗体(ab68915)
Key features and details
- Rabbit polyclonal to Myotilin
- Suitable for: IHC-P, WB
- Reacts with: Rat
- Isotype: IgG
概述
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产品名称
Anti-Myotilin抗体
参阅全部 Myotilin 一抗 -
描述
兔多克隆抗体to Myotilin -
宿主
Rabbit -
经测试应用
适用于: IHC-P, WBmore details -
种属反应性
与反应: Rat
预测可用于: Mouse, Cow, Human -
免疫原
Synthetic peptide conjugated to KLH derived from within residues 1 - 100 of Human Myotilin.
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阳性对照
- This antibody gave a positive signal in Rat Skeletal Muscle Tissue Lysate.
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常规说明
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle. -
存储溶液
pH: 7.40
Preservative: 0.02% Sodium azide
Constituent: PBS
Batches of this product that have a concentration < 1mg/ml may have BSA added as a stabilising agent. If you would like information about the formulation of a specific lot, please contact our scientific support team who will be happy to help. -
Concentration information loading...
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纯度
Immunogen affinity purified -
克隆
多克隆 -
同种型
IgG -
研究领域
相关产品
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Compatible Secondaries
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Isotype control
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab68915于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
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IHC-P |
Use a concentration of 5 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
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WB | (1) |
Use a concentration of 1 µg/ml. Detects a band of approximately 56 kDa (predicted molecular weight: 56 kDa).
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说明 |
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IHC-P
Use a concentration of 5 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol. |
WB
Use a concentration of 1 µg/ml. Detects a band of approximately 56 kDa (predicted molecular weight: 56 kDa). |
靶标
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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. -
组织特异性
Expressed in skeletal muscle (at protein level). Expressed in skeletal muscle, heart, bone marrow and thyroid gland. -
疾病相关
Defects in MYOT are the cause of limb-girdle muscular dystrophy type 1A (LGMD1A) [MIM:159000]. LGMD1A is an autosomal dominant degenerative myopathy with onset within a mean age of 28 years. LGMD1A is characterized by progressive skeletal muscle weakness of the hip and shoulder girdles, later progressing to include distal weakness, as well as a distinctive dysarthric pattern of speech. Affected muscle exhibits disorganization and streaming of the Z-line.
Defects in MYOT are the cause of myopathy myofibrillar myotylin-related (MFM-MYOT) [MIM:609200]. A neuromuscular disorder characterized by progressive skeletal muscle weakness greater distally than proximally, tight heel cords, hyporeflexia, cardiomyopathy and peripheral neuropathy in some patients. Affected muscle exhibits disorganization and streaming of the Z-line, presence of large hyaline structures, excessive accumulation of myotilin and other ectopically expressed proteins and prominent congophilic deposits.
Defects in MYOT are the cause of spheroid body myopathy (SBM) [MIM:182920]. SBM is an autosomal dominant form of myofibrillar myopathy (MFM), characterized by slowly progressing proximal muscle weakness and dysarthric nasal speech. There is no evidence of cardiomyopathy. Muscle biopsy shows spheroid bodies within the type I muscle fibers. -
序列相似性
Belongs to the myotilin/palladin family.
Contains 2 Ig-like C2-type (immunoglobulin-like) domains. -
细胞定位
Cell membrane > sarcolemma. Cytoplasm > cytoskeleton. Cytoplasm > myofibril > sarcomere > Z line. Sarcomeric, also localized to the sarcolemma. Colocalizes with MYOZ1 at the Z-lines in skeletal muscle. - Information by UniProt
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数据库链接
- Entrez Gene: 507884 Cow
- Entrez Gene: 9499 Human
- Entrez Gene: 58916 Mouse
- Entrez Gene: 291605 Rat
- Omim: 604103 Human
- SwissProt: Q9UBF9 Human
- SwissProt: Q9JIF9 Mouse
- Unigene: 84665 Human
see all -
别名
- 57 kDa cytoskeletal protein antibody
- LGMD 1 antibody
- LGMD1 antibody
see all
图片
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Anti-Myotilin antibody (ab68915) at 1 µg/ml + Skeletal Muscle (Rat) Tissue Lysate at 10 µg
Secondary
Goat polyclonal to Rabbit IgG - H&L - Pre-Adsorbed (HRP) at 1/3000 dilution
Performed under reducing conditions.
Predicted band size: 56 kDa
Observed band size: 56 kDa
Exposure time: 2 minutes -
IHC image of ab68915 staining in rat skeletal muscle formalin fixed paraffin embedded tissue section, performed on a Leica BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab68915, 5µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.
For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.
数据表及文件
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SDS download
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Datasheet download
文献 (3)
ab68915 被引用在 3 文献中.
- Li F et al. Deleting nebulin's C-terminus reveals its importance to sarcomeric structure and function and is sufficient to invoke nemaline myopathy. Hum Mol Genet 28:1709-1725 (2019). PubMed: 30689900
- Riedl I et al. Association of the ACTN3 R577X polymorphism with glucose tolerance and gene expression of sarcomeric proteins in human skeletal muscle. Physiol Rep 3:N/A (2015). PubMed: 25780092
- Lin X et al. Z-disc-associated, alternatively spliced, PDZ motif-containing protein (ZASP) mutations in the actin-binding domain cause disruption of skeletal muscle actin filaments in myofibrillar myopathy. J Biol Chem 289:13615-26 (2014). PubMed: 24668811