| Product Name | FKRP Antibody (H-128) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 368-495 mapping at the C-terminus of FKRP of human origin |
| Modification Notes | Fukutin-related protein (FKRP) is ubiquitously expressed, with highest expression in heart, skeletal muscle and placenta, and weakest expression in lung, liver, brain, kidney and pancreas. FKRP localizes to the medial Golgi apparatus through its N-terminal and transmembrane domains. It is a predicted glycosyltransferase protein that plays a role in ?-dystroglycan glycosylation. Mutations in FKRP cause various diseases including congenital muscular dystrophy 1C (MDC1C), limb-girdle muscular dystrophy type 2I (LGMD2I) and congenital muscular dystrophies (CMDs) with brain malformations and mental retardation. FKRP mutations may also cause muscle-eye-brain disease (MEB) and Walker-Warburg syndrome (WWS), disorders characterized by disruption of brain and eye structure in addition to muscular dystrophy. Mislocalization of FKRP from the Golgi apparatus is a potential result of mutations in FKRP. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of FKRP of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including canine, bovine and porcine: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 368-495 mapping at the C-terminus of FKRP of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 368-495 mapping at the C-terminus of FKRP of human origin |
| Entrez Gene | 79147 |
|---|
| Application Notes | recommended for detection of FKRP of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including canine, bovine and porcine: |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
