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WBS16 Antibody (N-term) Blocking peptide

Synthetic peptide

     
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Product Information
Primary Accession Q96I51
Clone Names 91113116
Additional Information
Gene ID 81554
Other Names Williams-Beuren syndrome chromosomal region 16 protein, RCC1-like G exchanging factor-like protein, WBSCR16
Target/Specificity The synthetic peptide sequence used to generate the antibody AP13957a was selected from the N-term region of WBS16. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.
Format Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed.
StorageMaintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.
PrecautionsThis product is for research use only. Not for use in diagnostic or therapeutic procedures.
Protein Information
Name RCC1L (HGNC:14948)
Function Guanine nucleotide exchange factor (GEF) for mitochondrial dynamin-related GTPase OPA1. Activates OPA1, by exchanging bound GDP for free GTP, and drives OPA1 and MFN1-dependent mitochondrial fusion (PubMed:28746876). Plays an essential role in mitochondrial ribosome biogenesis. As a component of a functional protein-RNA module, consisting of RCC1L, NGRN, RPUSD3, RPUSD4, TRUB2, FASTKD2 and 16S mitochondrial ribosomal RNA (16S mt-rRNA), controls 16S mt-rRNA abundance and is required for intra-mitochondrial translation of core subunits of the oxidative phosphorylation system (PubMed:27667664).
Cellular Location Mitochondrion membrane Mitochondrion inner membrane {ECO:0000250|UniProtKB:Q9CYF5}
Tissue Location Ubiquitous..
Research Areas
Citations (0)
citation

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Background

This gene encodes an RCC1-like G-exchanging factor. It isdeleted in Williams syndrome, a multisystem developmental disordercaused by the deletion of contiguous genes at 7q11.23. [provided byRefSeq].

References

Merla, G., et al. Hum. Genet. 110(5):429-438(2002)

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$ 277.78
Cat# BP13957a
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