Abcam, ab99957, Recombinant Human SBDS protein

Size: 100µg
Recombinant Human SBDS protein is a Human Full Length protein, in the 1 to 250 aa range, expressed in Escherichia coli, with >95%, suitable for SDS-PAGE, Mass Spec.
Key facts
Purity:>95% SDS-PAGE,
Expression system:Escherichia coli,
Tags:His tag N-Terminus,
Applications:Mass Spec, SDS-PAGESee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Biologically active:No,
Accession:Q9Y3A5,
Animal free:No,
Carrier free:No,
Species:Human,
Storage buffer:pH: 8Constituents: 20% Glycerol (glycerin, glycerine), 0.316% Tris HCl, 0.29% Sodium chloride, 0.0308% (R*,R*)-1,4-Dimercaptobutan-2,3-diol, 0.00292% EDTA

Product details:
Previously labelled as Shwachman Bodian-Diamond syndrome.

Properties and Storage Information:
Shipped at conditions-Blue Ice, Appropriate short-term storage conditions--20°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
SBDS also known as Shwachman-Bodian-Diamond syndrome protein participates in ribosome biogenesis. It has a molecular weight of approximately 29 kDa. SBDS is expressed in a variety of tissues with notable expression in the bone marrow and pancreas areas that require active ribosome synthesis. The protein works to assist in the assembly and maturation of the 60S ribosomal subunit associating closely with other ribosomal proteins and factors needed for proper cellular function.
Biological function summary
SBDS plays an essential role in the maturation of ribosomes and is part of the ribosomal large subunit assembly complex. It interacts with proteins such as EFL1 and Nucleophosmin highlighting its involvement in vital cellular processes. The protein contributes to protein synthesis machinery ensuring that ribosomal subunits are correctly formed and functional. SBDS is important for maintaining cellular homeostasis through its engagement in ribosome assembly.
Pathways
SBDS is involved in ribosome biogenesis a fundamental pathway for cell growth and division. This process is closely connected with proteins like eIF6 which cooperates with SBDS during the final stages of ribosomal subunit maturation. Additionally SBDS aligns with pathways related to cellular stress responses given its role in ensuring the integrity and function of ribosomes under conditions of cellular stress. These interactions demonstrate SBDS's importance in overarching cellular pathways that sustain life.
Mutations in the SBDS gene cause Shwachman-Diamond syndrome a rare congenital disorder that affects bone marrow function and pancreatic enzymes. The syndrome often results in neutropenia and pancreatic insufficiency manifesting through the disruption of ribosome biogenesis. Moreover research suggests SBDS might interact with proteins involved in other ribosomopathies connecting it indirectly to a spectrum of related disorders. Understanding SBDS’s role in these contexts provides insight into therapeutic targets and interventions for diseases involving ribosomal dysfunction.