MYBPC1 Rabbit pAb
- 产品详情
- 实验流程
Application
| WB, IHC-P, IHC-F, IF, E |
|---|---|
| Primary Accession | Q00872 |
| Other Accession | Q00872 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 128294 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human MYBPC1 |
| Epitope Specificity | 51-150/1141 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Belongs to the immunoglobulin superfamily. MyBP family. Contains 3 fibronectin type-III domains. Contains 7 Ig-like C2-type (immunoglobulin-like) domains. |
| SUBUNIT | Interacts with USP25 (isoform USP25m only); the interaction prevents proteasomal degradation of MYBPC1. |
| DISEASE | Defects in MYBPC1 are the cause of arthrogryposis, distal, type 1B (DA1B) [MIM:614335]. A form of distal arthrogryposis, a disease characterized by congenital joint contractures that mainly involve two or more distal parts of the limbs, in the absence of a primary neurological or muscle disease. Distal arthrogryposis type 1 is characterized largely by camptodactyly and clubfoot. Hypoplasia and/or absence of some interphalangeal creases is common. The shoulders and hips are less frequently affected. Note=Defects in MYBPC1 may be a cause of autosomal recessive lethal congenital contractural syndrome (LCCS), a severe, neonatally lethal form of arthrogryposis. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | MYBPC1 is a 1,141 amino acid protein that contains three fibronectin type-III domains and seven Ig-like C2-type domains. Existing as a member of the immunoglobulin superfamily, MYBPC1 functions as a thick filament-associated protein that localizes to striated muscle bands in vertebrae and is thought to modify the activity of select ATPases. Additionally, MYBPC1 may play a role in the modulation of muscle contraction and in the overall structural integrity of the cell. The gene encoding MYBPC1 maps to human chromosome 12, which encodes over 1,100 genes and comprises approximately 4.5% of the human genome. Chromosome 12 is associated with a variety of diseases and afflictions, including hypochondrogenesis, achondrogenesis, Kniest dysplasia, Noonan syndrome and Trisomy 12p, which causes facial developmental defects and seizure disorders. |
| Gene ID | 4604 |
|---|---|
| Other Names | CMYO16; CMYP16; LCCS4; MYBPCC; MYBPCS; MYOTREM; ssMyBP-C; 8030451F13Rik; MYPC1_HUMAN; MYBPC1; Slow MyBP-C; C-protein, skeletal muscle slow isoform; MYPC1_RAT; myosin binding protein C1; myosin-binding protein C, slow-type; slow skeletal-type muscle myosin-binding-protein C |
| Dilution | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | MYBPC1 |
|---|---|
| Synonyms | MYBPCS |
| Function | Thick filament-associated protein located in the crossbridge region of vertebrate striated muscle a bands. Slow skeletal protein that binds to both myosin and actin (PubMed:31025394, PubMed:31264822). In vitro, binds to native thin filaments and modifies the activity of actin-activated myosin ATPase. May modulate muscle contraction or may play a more structural role. |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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