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.
Applications:Suitable for use in Western Blot, Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemistry: 1:50-1:200Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：50ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid PBS, 0.1% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of SBDS. Species Crossreactivity: Human, mouse, rat
