Anti-DMD Antibody (CAB1411)
- SKU:
- CAB1411
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
| 抗体名: | Anti-DMD Antibody |
| 抗体コード: | CAB1411 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB IHC IF |
| 反応性: | Human, Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 346-635 of human DMD (NP_004007.1). |
| 申し込み: | WB IHC IF |
| 推奨希釈: | WB 1:200 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | Mouse heart |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 346-635 of human DMD (NP_004007.1). |
| 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: | NFWP VDSA PASS PQLS HDDT HSRI EHYA SRLA EMEN SNGS YLND SISP NESI DDEH LLIQ HYCQ SLNQ DSPL SQPR SPAQ ILIS LESE ERGE LERI LADL EEEN RNLQ AEYD RLKQ QHEH KGLS PLPS PPEM MPTS PQSP RDAE LIAE AKLL RQHK GRLE ARMQ ILED HNKQ LESQ LHRL RQLL EQPQ AEAK VNGT TVSS PSTS LQRS DSSQ PMLL RVVG SQTS DSMG EEDL LSPP QDTS TGLE EVME QLNN SFPS SRGH NVGS LFHM ADDL GRAM ESLV SVMT DEEG AE |
| 遺伝子ID: | 1756 |
| Uniprot: | P11532 |
| セルラーロケーション: | Cell junction, Cell membrane, Cytoplasm, Cytoplasmic side, Peripheral membrane protein, cytoskeleton, postsynaptic cell membrane, sarcolemma, synapse |
| 計算された分子量: | 57-72kDa/271kDa/425-426kDa |
| 観察された分子量: | 427kDa |
| 同義語: | DMD, BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272, MRX85 |
| バックグラウンド: | This gene spans a genomic range of greater than 2 Mb and encodes a large protein containing an N-terminal actin-binding domain and multiple spectrin repeats. The encoded protein forms a component of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton and the extracellular matrix. Deletions, duplications, and point mutations at this gene locus may cause Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), or cardiomyopathy. Alternative promoter usage and alternative splicing result in numerous distinct transcript variants and protein isoforms for this gene. |
| UniProt Protein Function: | dystrophin: Anchors the extracellular matrix to the cytoskeleton via F-actin. Ligand for dystroglycan. Component of the dystrophin- associated glycoprotein complex which accumulates at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems and has a structural function in stabilizing the sarcolemma. Also implicated in signaling events and synaptic transmission. Defects in DMD are the cause of Duchenne muscular dystrophy (DMD). DMD is the most common form of muscular dystrophy; a sex-linked recessive disorder. It typically presents in boys aged 3 to 7 year as proximal muscle weakness causing waddling gait, toe-walking, lordosis, frequent falls, and difficulty in standing up and climbing up stairs. The pelvic girdle is affected first, then the shoulder girdle. Progression is steady and most patients are confined to a wheelchair by age of 10 or 12. Flexion contractures and scoliosis ultimately occur. About 50% of patients have a lower IQ than their genetic expectations would suggest. There is no treatment. Defects in DMD are the cause of Becker muscular dystrophy (BMD). BMD resembles DMD in hereditary and clinical features but is later in onset and more benign. Defects in DMD are a cause of cardiomyopathy dilated X- linked type 3B (CMD3B); also known as X-linked dilated cardiomyopathy (XLCM). Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. 6 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Motility/polarity/chemotaxis; Cytoskeletal Chromosomal Location of Human Ortholog: Xp21.2 Cellular Component: filopodium membrane; cell surface; protein complex; costamere; syntrophin complex; Z disc; cytosol; cell-matrix junction; lipid raft; actin cytoskeleton; dystrophin-associated glycoprotein complex; postsynaptic membrane; cytoskeleton; plasma membrane; synapse; nucleus; sarcolemma; lateral plasma membrane; filopodium Molecular Function:protein binding; myosin binding; zinc ion binding; structural constituent of cytoskeleton; structural constituent of muscle; nitric-oxide synthase binding; actin binding; vinculin binding Biological Process: regulation of skeletal muscle contraction via regulation of the release of sequestered calcium ion; muscle development; extracellular matrix organization and biogenesis; regulation of heart rate; negative regulation of peptidyl-serine phosphorylation; peptide biosynthetic process; muscle filament sliding; muscle maintenance; cellular protein complex assembly; regulation of skeletal muscle contraction; muscle fiber development; positive regulation of neuron differentiation; cardiac muscle contraction Disease: Cardiomyopathy, Dilated, 3b; Muscular Dystrophy, Becker Type; Muscular Dystrophy, Duchenne Type |
| NCBI Summary: | This gene spans a genomic range of greater than 2 Mb and encodes a large protein containing an N-terminal actin-binding domain and multiple spectrin repeats. The encoded protein forms a component of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton and the extracellular matrix. Deletions, duplications, and point mutations at this gene locus may cause Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), or cardiomyopathy. Alternative promoter usage and alternative splicing result in numerous distinct transcript variants and protein isoforms for this gene. [provided by RefSeq, Dec 2016] |
| UniProt Code: | P11532 |
| NCBI GenInfo Identifier: | 313104240 |
| NCBI Gene ID: | 1756 |
| NCBI Accession: | P11532.3 |
| UniProt Secondary Accession: | P11532,P11531, P11530, |
| UniProt Related Accession: | P11532 |
| Molecular Weight: | 427kDa |
| NCBI Full Name: | Dystrophin |
| NCBI Synonym Full Names: | dystrophin |
| NCBI Official Symbol: | DMD |
| NCBI Official Synonym Symbols: | BMD; CMD3B; MRX85; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272 |
| NCBI Protein Information: | dystrophin |
| UniProt Protein Name: | Dystrophin |
| Protein Family: | Dystrophin |
| UniProt Gene Name: | DMD |
| UniProt Entry Name: | DMD_HUMAN |
 | Western blot analysis of extracts of mouse heart, using DMD antibody (CAB1411) 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 Enhanced Kit (RM00021)._Exposure time: 90s. |
 | Immunohistochemistry of paraffin-embedded rat heart using DMD antibody (CAB1411) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded mouse heart using DMD antibody (CAB1411) at dilution of 1:100 (40x lens). |
 | Immunofluorescence analysis of HeLa cells using DMD antibody (CAB1411) at dilution of 1:100. Blue: DAPI for nuclear staining. |
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