Alpha B Crystallin Recombinant Rabbit mAb
- 产品详情
- 实验流程
Application
| WB, IHC-P, IHC-F, IF, IP |
|---|---|
| Primary Accession | P02511 |
| Other Accession | P02511 |
| Host | Rabbit |
| Clonality | Recombinant |
| Calculated MW | 20159 Da |
| Physical State | Liquid |
| Immunogen | A synthesized peptide derived from human CRYAB |
| Epitope Specificity | 124-175 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 10mM phosphate buffered saline(pH 7.4) with 150mM sodium chloride, 0.05% BSA, 0.02% Proclin300 and 50% glycerol. |
| SIMILARITY | Belongs to the small heat shock protein (HSP20) family. |
| SUBUNIT | Heteropolymer composed of three CRYAA and one CRYAB subunits. Aggregates with homologous proteins, including the small heat shock protein HSPB1, to form large heteromeric complexes. Inter-subunit bridging via zinc ions enhances stability, which is crucial as there is no protein turn over in the lens. Interacts with HSPBAP1 and TTN/titin. |
| DISEASE | Defects in CRYAB are the cause of myopathy myofibrillar type 2 (MFM2) [MIM:608810]. A neuromuscular disorder that results in weakness of the proximal and distal limb muscles, weakness of the neck, velopharynx and trunk muscles, hypetrophic cardiomyopathy, and cataract in a subset of patients. [DISEASE] Defects in CRYAB are the cause of cataract posterior polar type 2 (CTPP2) [MIM:613763]. A subcapsular opacity, usually disk-shaped, located at the back of the lens. It can have a marked effect on visual acuity. [DISEASE] Defects in CRYAB are the cause of myopathy myofibrillar fatal infantile hypertonic alpha-B crystallin-related (MFMFIH-CRYAB) [MIM:613869]. MFMFIH-CRYAB is a muscular dystrophy with onset in the first weeks of life after a normal neonatal period. Affected infants show rapidly progressive muscular rigidity of the trunk and limbs associated with increasing respiratory difficulty resulting in death before age 3 years. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | May contribute to the transparency and refractive index of the lens. Has chaperone-like activity, preventing aggregation of various proteins under a wide range of stress conditions. In lens epithelial cells, stabilizes the ATP6V1A protein, preventing its degradation by the proteasome. |
| Gene ID | 1410 |
|---|---|
| Other Names | CMD1II; CRYA2; CTPP2; CTRCT16; HEL-S-101; HSPB5; MFM2; MFM2A; MFM2B; Crya-2; P23; AACRYA; CRYAB_HUMAN; CRYAB; Alpha(B)-crystallin; Heat shock protein beta-5 (HspB5); Heat shock protein family B member 5; Renal carcinoma antigen NY-REN-27; Rosenthal fiber component; CRYAB_MOUSE; CRYAB_RAT; crystallin alpha B; crystallin, alpha B |
| Dilution | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:50-200,IF=1:100-500,IP=1:20-50 |
| 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 | CRYAB (HGNC:2389) |
|---|---|
| Synonyms | CRYA2, HSPB5 |
| Function | May contribute to the transparency and refractive index of the lens. Has chaperone-like activity, preventing aggregation of various proteins under a wide range of stress conditions. In lens epithelial cells, stabilizes the ATP6V1A protein, preventing its degradation by the proteasome (By similarity). |
| Cellular Location | Cytoplasm. Nucleus Secreted. Lysosome {ECO:0000250|UniProtKB:P23927}. Note=Translocates to the nucleus during heat shock and resides in sub-nuclear structures known as SC35 speckles or nuclear splicing speckles (PubMed:19464326). Localizes at the Z- bands and the intercalated disk in cardiomyocytes (PubMed:28493373) Can be secreted; the secretion is dependent on protein unfolding and facilitated by the cargo receptor TMED10; it results in protein translocation from the cytoplasm into the ERGIC (endoplasmic reticulum- Golgi intermediate compartment) followed by vesicle entry and secretion (PubMed:32272059). |
| Tissue Location | Lens as well as other tissues (PubMed:2387586, PubMed:838078). Expressed in myocardial tissue (PubMed:28493373) |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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