SBDS Human; Shwachman-Bodian-Diamond Syndrome Human Recombinant; SDS; SWDS; Shwachman-Bodian-Diamond syndrome; Ribosome Maturation protein SBDS

For Research Use Only. Not for use in diagnostic procedures.

E Coli

Greater than 95% as determined by SDS-PAGE.

The SBDS protein solution (0.5mg/1ml) is formulated in 20mM Tris-HCl buffer (pH8.0) 2mM DTT, 50mM NaCl, 0.1mM EDTA, and 20% glycerol.
Sterile Filtered clear solution.

Store at 4 degree C if entire vial will be used within 2-4 weeks. Store, frozen at -20 degree C for longer periods of time.Please avoid freeze thaw cycles.

Small volumes of SBDS recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Description: SBDS produced in E Coli is a single, non-glycosylated polypeptide chain containing 270 amino acids (1-250a.a.) and having a molecular mass of 30.9kDa.SBDS is fused to a 20 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Introduction: SBDS is part of an extremely preserved protein family which exists from archaea to vertebrates and plants. SBDS protein functions in RNA metabolism and has a role in the biogenesis of the 60S ribosomal subunit and translational activation of ribosomes. Shwachman-Diamond syndrome is a rare autosomal recessive disorder produced by mutations in the SBDS gene.

RECOMBINANT & NATURAL PROTEINS; Recombinant Proteins

28,764 Da

Shwachman-Bodian-Diamond syndrome

ribosome maturation protein SBDS

Ribosome maturation protein SBDS

SBDS_HUMAN

This gene encodes a member of a highly conserved protein family that exists from archaea to vertebrates and plants. The encoded protein may function in RNA metabolism. Mutations within this gene are associated with Shwachman-Bodian-Diamond syndrome. An alternative transcript has been described, but its biological nature has not been determined. This gene has a closely linked pseudogene that is distally located. [provided by RefSeq, Jul 2008]

SBDS: Required for the assembly of mature ribosomes and ribosome biogenesis. Together with EFTUD1, triggers the GTP- dependent release of EIF6 from 60S pre-ribosomes in the cytoplasm, thereby activating ribosomes for translation competence by allowing 80S ribosome assembly and facilitating EIF6 recycling to the nucleus, where it is required for 60S rRNA processing and nuclear export. Required for normal levels of protein synthesis. May play a role in cellular stress resistance. May play a role in cellular response to DNA damage. May play a role in cell proliferation. Defects in SBDS are the cause of Shwachman-Diamond syndrome (SDS). SDS is an autosomal recessive disorder characterized by pancreatic exocrine insufficiency, hematologic dysfunction, and skeletal abnormalities. Belongs to the SDO1/SBDS family.

Protein type: Nucleolus

Chromosomal Location of Human Ortholog: 7q11.21

Cellular Component: nucleoplasm; spindle pole; cytoplasm; nucleolus; nucleus

Molecular Function: rRNA binding; protein binding; microtubule binding; ribosome binding

Biological Process: bone marrow development; cell proliferation; leukocyte chemotaxis; ribosomal large subunit biogenesis and assembly; mitotic spindle stabilization; inner cell mass cell proliferation; rRNA processing; mature ribosome assembly; bone mineralization

Disease: Shwachman-diamond Syndrome; Aplastic Anemia

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