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Rabbit Anti-Desmuslin  antibody (bs-8555R)  
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產品編號 bs-8555R
英文名稱 Rabbit Anti-Desmuslin  antibody
中文名稱 中間絲蛋白β-synemin抗體
別    名 Desmuslin; DMN; SYN; Synemin alpha; Synemin beta; Synemin, intermediate filament protein; SYNM; KIAA0353; synemin isoform A; SYNEM_HUMAN.  
Specific References  (4)     |     bs-8555R has been referenced in 4 publications.
[IF=15.887] Weihl, Conrad C.. et al. Loss of function variants in DNAJB4 cause a myopathy with early respiratory failure. ACTA NEUROPATHOL. 2022 Oct;:1-17  WB ;  Mouse.  
[IF=6.39] Bengoechea, Rocio, et al. "Myofibrillar disruption and RNA binding protein aggregation in a mouse model of limb girdle muscular dystrophy 1D." Human Molecular Genetics (2015): ddv363.  WB ;  Mouse.  
[IF=6.183] Jipeng Jiang. et al. 3D printing collagen/heparin sulfate scaffolds boost neural network reconstruction and motor function recovery after traumatic brain injury in canine. Biomater Sci-Uk. 2020 Nov;8(22):6362-6374  IF ;  Dog.  
[IF=4.902] Bengoechea et al. Myofibrillar disruption and RNA-binding protein aggregation in a mouse model of limb-girdle muscular dystrophy 1D. (2015) Hum.Mol.Genet. 24:6588-602  WB ;  Mouse.  
研究領域 細胞生物  信號轉導  細胞外基質  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Rat (predicted: Human,Mouse,Rabbit,Pig,Cow)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:200-800
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 172kDa
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Desmuslin: 501-650/1565 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 The protein encoded by this gene is an intermediate filament (IF) family member. IF proteins are cytoskeletal proteins that confer resistance to mechanical stress and are encoded by a dispersed multigene family. This protein has been found to form a linkage between desmin, which is a subunit of the IF network, and the extracellular matrix, and provides an important structural support in muscle. Two alternatively spliced variants encoding different isoforms have been described for this gene. [provided by RefSeq, Jul 2008].

Function:
Type-VI intermediate filament (IF) which plays an important cytoskeletal role within the muscle cell cytoskeleton. It forms heteropolymeric IFs with desmin and/or vimentin, and via its interaction with cytoskeletal proteins alpha-dystrobrevin, dystrophin, talin-1, utrophin and vinculin, is able to link these heteropolymeric IFs to adherens-type junctions, such as to the costameres, neuromuscular junctions, and myotendinous junctions within striated muscle cells.

Subunit:
Interacts with GFAP and VIM (By similarity). Isoform 1 interacts with TLN1 and VCL. Isoform 2 interacts with DES and DTNA. Isoform 1 and isoform 2 interact with DMD and UTRN.

Subcellular Location:
Cytoplasm, cytoskeleton. Cell junction, adherens junction.

Tissue Specificity:
Isoform 2 is strongly detected in adult heart, fetal skeletal muscles and fetal heart. Isoform 1 is weakly detected in fetal heart and also in fetal skeletal muscle. Isoform 1 and isoform 2 are detected in adult bladder (at protein level). The mRNA is predominantly expressed in heart and muscle with some expression in brain which may be due to tissue-specific isoforms.

Similarity:
Belongs to the intermediate filament family.

SWISS:
O15061

Gene ID:
23336

Database links:

Entrez Gene: 23336 Human

Entrez Gene: 233335 Mouse

Entrez Gene: 308709 Rat

Omim: 606087 Human

SwissProt: O15061 Human

SwissProt: Q70IV5 Mouse

Unigene: 207106 Human

Unigene: 60526 Mouse

Unigene: 46362 Rat



產品圖片
Paraformaldehyde-fixed, paraffin embedded (Rat skeletal muscle); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Desmuslin) Polyclonal Antibody, Unconjugated (bs-8555R) at 1:400 overnight at 4°C, followed by a conjugated Goat Anti-Rabbit IgG antibody (bs-0295G-FITC) for 90 minutes, and DAPI for nuclei staining.
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