Dystrophin Antibody (YA3224)
(Synonyms: BMD; CMD3B; DMD; Dystrophin)Dystrophin Antibody (YA3224) is a Rabbit-derived and non-conjugated IgG monoclonal antibody, targeting to Dystrophin.
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Host:
Rabbit
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Isotype:
IgG
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Application:
WB
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Reactivity :
Human, Mouse, Rat
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Formulation:
Supplied in rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
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Conjugation:
Non-conjugated
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Applications
| Application |
WB
WB: Western Blot
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|---|---|
| Dilution Ratio | 1:500-1:1000 |
Product Details
Dystrophin Antibody (YA3224) is a Rabbit-derived and non-conjugated IgG monoclonal antibody, targeting to Dystrophin.
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Host Rabbit
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Clonality Recombinant,Monoclonal
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Species ReactivityHuman, Mouse, Rat
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Observed Molecular WeightObserved band size: 427 kDa
Note: Due to possible protein modifications or aggregation, the molecular weight should be confirmed by actual measurement, and the predicted value is for reference only.
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Calculated Molecular Weight Predicted band size: 427 kDa
Entrez Gene: 1756 Human ; 13405 Mouse ;
SwissProt: P11532 Human ; P11531 Mouse ; P11530 Rat
OMIM: 310200 Human
A synthesized peptide derived from human Dystrophin aa3611-3685.
Endogenous
Affinity Chromatography
Non-conjugated
Unmodified
IgG
Product Properties
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Appearance
Solution
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Formulation
Supplied in rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
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Storage & Stability
Stored at -20°C for 1 year. Avoid repeated freeze / thaw cycles.
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Shipping
Shipping with blue ice.
Background
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Function
Dystrophin is a rod-shaped cytoskeletal protein that connects the intercellular cytoskeleton to the extracellular matrix through an actin-associated glycoprotein complex. This property enables Dystrophin to play a role in sarcolemmal stability during muscle contraction and prevent contraction damage. Dystrophin also mediates cell signaling, such as mechanotransduction and cell adhesion. However, Dystrophin deficiency or mutation (producing internally truncated Dystrophin) will become a predisposing factor for Duchenne muscular dystrophy (DMD) . In the muscle tissue of mild and asymptomatic patients, a large number of alternative Dystrophin splicing protein products can be detected. Currently, the methods for restoring Dystrophin mutations rely on virus-mediated restoration or exon skipping. Exon skipping uses antisense oligonucleotides to induce alternative splicing, bypassing the mutated exon to restore the protein reading frame, and converting DMD mutations to Becker muscular dystrophy (BMD) protein mutations.Dystrophin-related proteins can be divided into three groups (according to subcellular localization) : (1) α-dystroglycan located outside the cell; (2) β-dystroglycan, sarcoglycans, and sarcospan located on the plasma membrane; (3) Dystrophin, dystrobrevin, syntrophins, and neuronal nitric oxide synthase located inside the cell. Dystrophin has four major functional domains: the actin-binding amino-terminal domain (ABD1), the central rod domain, the cysteine-rich domain, and the carboxyl terminus. Dystrophin interacts with tubulin and acidic actin filaments through the rod domain, so Dystrophin mutations not only lead to progressive loss of muscle tissue and function, but also cause cardiomyopathy[1][2][3].
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Subcellular Localization
Cell membrane, sarcolemma; Peripheral membrane protein; Cytoplasmic side; Cytoplasm, cytoskeleton; Postsynaptic cell membrane
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Expression
Tissue_specificity:This gene is expressed in myofibrils and accumulates in the sarcoplasm of the sarcolemma. It is expressed in the brain, muscle, kidneys, lungs, and testes. Multiple transcriptotypes are present in most tissues. Isotype 15: Detected only in the heart and liver, but also expressed in brain, testicular, and liver cancer cells. -
Isoforms & Post-Translational Modification
P11532 has 17 isomers: P11532-1: 426778 Da (predicted); P11532-4: 425668 Da (predicted); P11532-11: 426029 Da (predicted); P11532-2: 271419 Da (predicted); P11532-3: 271068 Da (predicted); P11532-12: 141399 Da (predicted); P11532-13: 128978 Da (predicted); P11532-14: 143215 Da (predicted); P11532-15: 141774 Da (predicted); P11532-16: 130794 Da (predicted); P11532-17: 109970 Da (predicted); P11532-7: 70375 Da (predicted); P11532-8: 68934 Da (predicted); P11532-6: 72191 Da (predicted); P11532-5: 70750 Da (predicted); P11532-9: 59769 Da (predicted); P11532-18: 39254 Da (predicted).
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Subunit
Interacts with the syntrophins SNTA1, SNTB1, SNTB2, SNTG1 and SNTG2 (PubMed:7844150, PubMed:8576247). Interacts with KRT19 (PubMed:16000376).
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SwissProt ID
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Synonyms
BMD; CMD3B; DMD; Dystrophin
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Research Field
Signal Transduction
Documentation
References
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