Shwachman Bodian Diamond syndrome (SBDS) (1-250) Mouse Monoclonal Antibody [Clone ID: AT1E8]

Specifications

Product Data
Clone Name	AT1E8
Applications	ELISA, IF, WB
Recommend Dilution	ELISA. Western blot: 1/3000. Immunocytochemistry / Immunoflourescence.
Reactivity	Human
Host	Mouse
Clonality	Monoclonal
Immunogen	Recombinant human SBDS (1-250aa) purified from E. coli
Specificity	This antibody detects recombinant Human SBDS (1-250aa) Other species not tested.
Formulation	PBS, pH 7.4 containing 0.02% Sodium Azide and 10% Glycerol State: Purified State: Liquid purified Ig fraction
Concentration	lot specific
Purification	Protein-G affinity chromatography
Conjugation	Unconjugated
Storage Condition	Store undiluted at 2-8°C for up to two weeks or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing.
Gene Name	SBDS ribosome assembly guanine nucleotide exchange factor
Database Link	Entrez Gene 51119 Human UniProt ID Q9Y3A5
Background	The Shwachman-Bodian-Diamond syndrome (SBDS) is 249 amino acid ribosome maturation protein. The precise function of the SBDS protein is not known but it appears to play an important role in actin cytoskeletal function and mitotic spindle stabilization. Also, SBDS is required for the assembly of mature ribosomes and ribosome biogenesis. Mutations in the SBDS gene cause Shwachman-Diamond syndrome (SDS). SDS is an autosomal recessive disorder with clinical features that include pancreatic exocrine insufficiency, haematological dysfunction and skeletal abnormalities.
Synonyms	Shwachman-Bodian-Diamond syndrome protein
Reference Data

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