Mouse anti-Human Shwachman-Bodian-Diamond Syndrome Monoclonal Antibody | anti-SBDS antibody

Mouse Anti Human Shwachman-Bodian-Diamond Syndrome

Cat. Num. AAA140428

• Gene Names: SBDS; SDS; SWDS; CGI-97
• Reactivity: Human
• Purity: SBDS antibody was purified from mouse ascitic fluids by protein-G affinity chromatography
• Synonyms: Shwachman-Bodian-Diamond Syndrome; Monoclonal Antibody; Mouse Anti Human Shwachman-Bodian-Diamond Syndrome; SBDS Antibody; Mouse Anti Human; SDS; SWDS; Shwachman-Bodian-Diamond syndrome; Ribosome Maturation protein SBDS; anti-SBDS antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: Mouse IgG2b heavy chain and kappa light chain
• Clone Number: PAT1E8AT
• Purity/Purification: SBDS antibody was purified from mouse ascitic fluids by protein-G affinity chromatography
• Form/Format: 1mg/ml containing PBS, pH-7.4, 0.02% sodium azide and 10% Glycerol
• Sequence Length: 250

• Immunogen: Anti-human SBDS mAb, is derived from hybridization of mouse FO myeloma cells with spleen cells from BALB/c mice immunized with recombinant human SBDS amino acids 1-250 purified from E Coli.
• Physical Appearance: Sterile filtered colorless solution
• Preparation and Storage: For periods up to 1 month store at 4°C, for longer periods of time, store at -20°C.; Prevent freeze thaw cycles.; 12 months at -20°C. 1 month at 4°C

Related Product Information for anti-SBDS antibody

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.

Product Categories/Family for anti-SBDS antibody

Monoclonal/Polyclonal Antibodies; Monoclonal Antibodies

NCBI and Uniprot Product Information

• NCBI GI #: 41350825
• NCBI GeneID: 51119
• NCBI Official Full Name: Shwachman-Bodian-Diamond syndrome
• NCBI Official Synonym Full Names: Shwachman-Bodian-Diamond syndrome
• NCBI Official Symbol: SBDS
• NCBI Official Synonym Symbols: SDS; SWDS; CGI-97
• NCBI Protein Information: ribosome maturation protein SBDS
• UniProt Protein Name: Ribosome maturation protein SBDS
• Protein Family: Ribosome maturation protein
• UniProt Gene Name: SBDS
• UniProt Entry Name: SBDS_HUMAN

NCBI Description

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]

Uniprot Description

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; inner cell mass cell proliferation; mitotic spindle stabilization; bone mineralization; mature ribosome assembly; rRNA processing

Disease: Shwachman-diamond Syndrome; Aplastic Anemia