| Product Name | SBDS Antibody (FL-250) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 1-250 representing full length SBDS of human origin |
| Modification Notes | The 249 amino acid Shwachman-Bodian-Diamond syndrome (SBDS) protein belongs to the UPF0023 family. SBDS is widely expressed and may be involved in RNA metabolism. SBDS contains a C-terminal domain, a central domain and an N-terminal domain. The C-terminal domain has a ferredoxin-like fold and is structurally homologous with known RNA-binding domains. The central domain contains a three-helical bundle. The N-terminal domain consists of a three-dimensional a/b fold and is the most frequent target of disease-linked mutations. Mutations in the SBDS gene cause Shwachman-Diamond syndrome (SDS), an autosomal recessive marrow failure disorder marked by hematologic dysfunction, skeletal abnormalities and pancreatic exocrine insufficiency. SDS is also characterized by an increased risk of leukemia and myelodysplasia in as many as one third of affected individuals. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of SBDS of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine, porcine and avian: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 1-250 representing full length SBDS of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 1-250 representing full length SBDS of human origin |
| Entrez Gene | 51119 |
|---|
| Application Notes | recommended for detection of SBDS of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine, porcine and avian: |
|---|
| 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. |
