BS8071

antibody from Bioworld Technology, Inc
Targeting: SBDS CGI-97, FLJ10917, SDS, SWDS
Provider product page for BS8071
Western blot
Immunohistochemistry

Antibody data

Product number
BS8071 - Provider product page
Provider
Bioworld Technology, Inc
Product name
SBDS polyclonal antibody
Antibody type
Polyclonal
Antigen
Recombinant full length Human SBDS.
Description
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.
Reactivity
Human, Mouse, Rat
Host
Rabbit
Isotype
IgG
Vial size
100ul
Concentration
1 mg/ml
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
SBDS protein structure - BS8071 shown in red.