This antibody gave a positive signal in Human, Mouse and Rat Skeletal Muscle tissue lysates.
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 Note: 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.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 µg/ml. Detects a band of approximately 223 kDa (predicted molecular weight: 223 kDa).
Use a concentration of 1 µg/ml.
疾病相关Defects in MYH3 are the cause of distal arthrogryposis type 2A (DA2A) [MIM:193700]; also known as Freeman-Sheldon syndrome (FSS). Distal arthrogryposis is a clinically and genetically heterogeneous group of disorders characterized by bone anomalies and joint contractures of the hands and feet, causing medially overlapping fingers, clenched fists, ulnar deviation of fingers, camptodactyly and positional foot deformities. It is a disorder of primary limb malformation without primary neurologic or muscle disease. DA2A is the most severe form of distal arthrogryposis. Affected individuals have contractures of the orofacial muscles, characterized by microstomia with pouting lips, H-shaped dimpling of the chin, deep nasolabial folds, and blepharophimosis. Dysphagia, failure to thrive, growth deficit, and life-threatening respiratory complications (caused by structural anomalies of the oropharynx and upper airways) are frequent. Inheritance is autosomal dominant. Defects in MYH3 are the cause of distal arthrogryposis type 2B (DA2B) [MIM:601680]; also known as Sheldon-Hall syndrome (SHS) or arthrogryposis multiplex congenita distal type 2B (AMCD2B). DA2B is a form of inherited multiple congenital contractures. Affected individuals have vertical talus, ulnar deviation in the hands, severe camptodactyly, and a distinctive face characterized by a triangular shape, prominent nasolabial folds, small mouth and a prominent chin. DA2B is the most common of the distal arthrogryposis syndromes. It is similar to DA2A but the facial contractures are less dramatic.
序列相似性Contains 1 IQ domain. Contains 1 myosin head-like domain.
发展阶段Abundantly present in fetal skeletal muscle and not present or barely detectable in heart and adult skeletal muscle.
结构域The rodlike tail sequence is highly repetitive, showing cycles of a 28-residue repeat pattern composed of 4 heptapeptides, characteristic for alpha-helical coiled coils. Each myosin heavy chain can be split into 1 light meromyosin (LMM) and 1 heavy meromyosin (HMM). It can later be split further into 2 globular subfragments (S1) and 1 rod-shaped subfragment (S2).
细胞定位Cytoplasm > myofibril. Thick filaments of the myofibrils.
IHC image of Fast Myosin Skeletal Heavy chain staining in Human normal 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 ab124205, 1µg/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.
Western blot - Anti-Fast Myosin Skeletal Heavy chain antibody (ab124205)
All lanes : Anti-heavy chain Myosin antibody (ab124205) at 1 µg/ml
Lane 1 : Skeletal Muscle (Human) Tissue Lysate - fetal normal tissue Lane 2 : Human heart tissue lysate - total protein (ab29431) Lane 3 : Human skeletal muscle tissue lysate - total protein (ab29330) Lane 4 : Human small intestine tissue lysate - total protein (ab29276) Lane 5 : Heart (Human) Tissue Lysate - fetal normal tissue Lane 6 : Human skin tissue lysate - total protein (ab30166)
Lysates/proteins at 10 µg per lane.
Secondary Goat Anti-Rabbit IgG H&L (HRP) (ab97051) at 1/10000 dilution developed using the ECL technique
Performed under reducing conditions.
Predicted band size : 223 kDa Observed band size : 223 kDa Additional bands at : 190 kDa,40 kDa,50 kDa. We are unsure as to the identity of these extra bands.
Exposure time : 30 seconds
Anti-heavy chain Myosin antibody (ab124205)参考文献
This product has been referenced in:
Sakellariou GK et al. Neuron-specific expression of CuZnSOD prevents the loss of muscle mass and function that occurs in homozygous CuZnSOD-knockout mice. FASEB J28:1666-81 (2014).
Read more (PubMed: 24378874) »