Shwachman-Bodian-Diamond syndrome; SBDS; CGI-97; FLJ10917; SDS; SWDS

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

Polyclonal

IgG

Rabbit

Antigen Affinity Purified

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-SBDS antibody 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.

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

28,764 Da[Similar Products]

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; bone mineralization; rRNA processing; mature ribosome assembly

Disease: Shwachman-diamond Syndrome; Aplastic Anemia

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